Libre Pathology - User contributions [en]

Pseudoangiomatous stromal hyperplasia

2019-03-26T01:19:39Z

Brigitte: /* Images */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Pseudoangiomatous_stromal_hyperplasia_-_high_mag.jpg
| Width =
| Caption = Pseudoangiomatous stromal hyperplasia. [[H&E stain]].
| Synonyms =
| Micro = abundant breast stroma, small complex inter-anastomosing (blood vessel/capillary-like) channels
| Subtypes =
| LMDDx = [[fibroadenoma]], low-grade [[angiosarcoma]]
| Stains = Bcl-2 +ve, vimentin +ve, CD34 +ve, SMMHC +ve, CD31 -ve, factor VIII -ve
| IHC =
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = [[breast]]
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs = +/-breast mass
| Symptoms =
| Prevalence = uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = benign
| Other =
| ClinDDx = [[fibroadenoma]]
| Tx = followup or excision
}}
'''Pseudoangiomatous stromal hyperplasia''', abbreviated ''PASH'', is benign lesion is [[breast pathology]].

It is also known as '''nodular myofibroblastic stromal hyperplasia of the mammary gland'''.<ref name=pmid12199757>{{Cite journal | last1 = Leon | first1 = ME. | last2 = Leon | first2 = MA. | last3 = Ahuja | first3 = J. | last4 = Garcia | first4 = FU. | title = Nodular myofibroblastic stromal hyperplasia of the mammary gland as an accurate name for pseudoangiomatous stromal hyperplasia of the mammary gland. | journal = Breast J | volume = 8 | issue = 5 | pages = 290-3 | month = | year = | doi = | PMID = 12199757 }}</ref>

==General==
*Benign lesion.
*Thought to arise due to myofibroblast abnormality - though not well understood.<ref name=pmid7872425>{{cite journal |author=Powell CM, Cranor ML, Rosen PP |title=Pseudoangiomatous stromal hyperplasia (PASH). A mammary stromal tumor with myofibroblastic differentiation |journal=Am. J. Surg. Pathol. |volume=19 |issue=3 |pages=270–7 |year=1995 |month=March |pmid=7872425 |doi= |url=}}</ref>
*An incidental finding ''or'' occasionally a mass.<ref name=pmid21862488>{{Cite journal | last1 = Drinka | first1 = EK. | last2 = Bargaje | first2 = A. | last3 = Erşahin | first3 = ÇH. | last4 = Patel | first4 = P. | last5 = Salhadar | first5 = A. | last6 = Sinacore | first6 = J. | last7 = Rajan | first7 = P. | title = Pseudoangiomatous stromal hyperplasia (PASH) of the breast: a clinicopathological study of 79 cases. | journal = Int J Surg Pathol | volume = 20 | issue = 1 | pages = 54-8 | month = Feb | year = 2012 | doi = 10.1177/1066896911418643 | PMID = 21862488 }}</ref>
*May be suspicious on imaging ([[BI-RADS]] 4 or 5).<ref name=pmid19097540>{{Cite journal | last1 = Wieman | first1 = SM. | last2 = Landercasper | first2 = J. | last3 = Johnson | first3 = JM. | last4 = Ellis | first4 = RL. | last5 = Wester | first5 = SM. | last6 = Lambert | first6 = PJ. | last7 = Ross | first7 = LA. | title = Tumoral pseudoangiomatous stromal hyperplasia of the breast. | journal = Am Surg | volume = 74 | issue = 12 | pages = 1211-4 | month = Dec | year = 2008 | doi = | PMID = 19097540 }}</ref>
*May be followed (expectant management) or (surgically) excised.<ref name=pmid20887819>{{Cite journal | last1 = Jaunoo | first1 = SS. | last2 = Thrush | first2 = S. | last3 = Dunn | first3 = P. | title = Pseudoangiomatous stromal hyperplasia (PASH): a brief review. | journal = Int J Surg | volume = 9 | issue = 1 | pages = 20-2 | month = | year = 2011 | doi = 10.1016/j.ijsu.2010.09.005 | PMID = 20887819 }}</ref>

==Gross==
Features:<ref name=pmid7872425>{{cite journal |author=Powell CM, Cranor ML, Rosen PP |title=Pseudoangiomatous stromal hyperplasia (PASH). A mammary stromal tumor with myofibroblastic differentiation |journal=Am. J. Surg. Pathol. |volume=19 |issue=3 |pages=270–7 |year=1995 |month=March |pmid=7872425 |doi= |url=}}</ref>
*May form mass.
**Grey-white & firm.
**Well-circumscribed borders.

==Microscopic==
Features:<ref name=pmid3949338>{{cite journal |author=Vuitch MF, Rosen PP, Erlandson RA |title=Pseudoangiomatous hyperplasia of mammary stroma |journal=Hum. Pathol. |volume=17 |issue=2 |pages=185–91 |year=1986 |month=February |pmid=3949338 |doi= |url=}}</ref><ref name=pmid18084246>{{Cite journal | last1 = Ferreira | first1 = M. | last2 = Albarracin | first2 = CT. | last3 = Resetkova | first3 = E. | title = Pseudoangiomatous stromal hyperplasia tumor: a clinical, radiologic and pathologic study of 26 cases. | journal = Mod Pathol | volume = 21 | issue = 2 | pages = 201-7 | month = Feb | year = 2008 | doi = 10.1038/modpathol.3801003 | PMID = 18084246 }}</ref>
*Abundant breast stromal.
*Small, complex, inter-anastomosing (blood vessel/capillary-like) channels - '''key feature'''.
**''Pseudoangiomatous'' = blood vessel-like.

Notes:
*May mimic angiosarcoma at low power; PASH may have the same architecture but lack nuclear atypia.
*Occasionally has mitotic activity - ''proliferative PASH''.

DDx:
*[[Angiosarcoma]].
*[[Fibroadenoma]].

===Images===
<gallery>
Image:Pseudoangiomatous stromal hyperplasia -a- low mag.jpg | PASH - low mag. (WC/Nephron)
Image:Pseudoangiomatous stromal hyperplasia -a- intermed mag.jpg | PASH - intermed. mag. (WC/Nephron)
Image:Pseudoangiomatous stromal hyperplasia -a2- intermed mag.jpg | PASH - intermed. mag. (WC/Nephron)
Image:Pseudoangiomatous stromal hyperplasia -a- high mag.jpg | PASH - high mag. (WC/Nephron)
</gallery>
<gallery>
Image:Pseudoangiomatous stromal hyperplasia - intermed mag.jpg | PASH - intermed. mag. (WC/Nephron)
Image:Pseudoangiomatous stromal hyperplasia - high mag.jpg | PASH - high mag. (WC/Nephron)
Image:Pseudoangiomatous stromal hyperplasia - very high mag.jpg | PASH - very high mag. (WC/Nephron)
</gallery>
www:
<ref name=pmid18084246>{{Cite journal | last1 = Ferreira | first1 = M. | last2 = Albarracin | first2 = CT. | last3 = Resetkova | first3 = E. | title = Pseudoangiomatous stromal hyperplasia tumor: a clinical, radiologic and pathologic study of 26 cases. | journal = Mod Pathol | volume = 21 | issue = 2 | pages = 201-7 | month = Feb | year = 2008 | doi = 10.1038/modpathol.3801003 | PMID = 18084246 }}</ref>

==IHC==
Findings:<ref name=pmid7872425/>
*CD34 +ve.
*Vimentin +ve.
*Bcl-2 +ve.<ref name=pmid21843705/><ref>URL: [http://surgpathcriteria.stanford.edu/breast/pash/printable.html http://surgpathcriteria.stanford.edu/breast/pash/printable.html]. Accessed on: 28 April 2012.</ref>
*Factor VIII -ve.
*CD31 -ve.<ref name=pmid21843705>{{Cite journal | last1 = Baker | first1 = M. | last2 = Chen | first2 = H. | last3 = Latchaw | first3 = L. | last4 = Memoli | first4 = V. | last5 = Ornvold | first5 = K. | title = Pseudoangiomatous stromal hyperplasia of the breast in a 10-year-old girl. | journal = J Pediatr Surg | volume = 46 | issue = 8 | pages = e27-31 | month = Aug | year = 2011 | doi = 10.1016/j.jpedsurg.2011.04.063 | PMID = 21843705 }}</ref>

==See also==
*[[Breast pathology]].
*[[Fibroadenoma]].

==References==
{{Reflist|2}}

[[Category:Breast pathology]]
[[Category:Diagnosis]]

Neuroendocrine tumour of the pancreas

2019-03-03T20:21:02Z

Brigitte: /* WHO classification of 2017 */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Pancreatic neuroendocrine tumour - 2 -- high mag.jpg
| Width =
| Caption = Pancreatic neuroendocrine tumour. [[H&E stain]].
| Synonyms = pancreatic islet cell tumour (obsolete term)
| Micro = nests of cells or cords, stippled chromatin, moderate quantity of cytoplasm, +/-hyaline globules
| Subtypes =
| LMDDx = [[solid pseudopapillary neoplasm]], [[acinar cell carcinoma]], [[invasive ductal carcinoma of the pancreas]]
| Stains =
| IHC = chromogranin +ve, synaptophysin +ve, insulin +/-ve, glucagon +/-ve, gastrin +/-ve, somatostatin +/-ve
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = [[pancreas]]
| Assdx =
| Syndromes = [[Multiple endocrine neoplasia I]], [[von Hippel-Lindau disease]], [[neurofibromatosis type 1]]
| Clinicalhx =
| Signs =
| Symptoms = dependent on subtype
| Prevalence = uncommon
| Bloodwork =
| Rads = pancreatic mass
| Endoscopy =
| Prognosis =
| Other =
| ClinDDx = [[invasive ductal carcinoma of the pancreas]], other pancreatic tumours
| Tx =
}}
'''Neuroendocrine tumour of the pancreas''', also '''pancreatic neuroendocrine tumour''', is a relatively uncommon tumour.

It may be abbreviated '''PanNET'''.<ref name=pmid22198808/>

Previously, it was referred to as '''pancreatic islet cell tumour''' or '''islet cell tumour'''; these terms are now considered to be outdated.<ref name=pmid22198808>{{Cite journal | last1 = Burns | first1 = WR. | last2 = Edil | first2 = BH. | title = Neuroendocrine Pancreatic Tumors: Guidelines for Management and Update. | journal = Curr Treat Options Oncol | volume = | issue = | pages = | month = Dec | year = 2011 | doi = 10.1007/s11864-011-0172-2 | PMID = 22198808 }}</ref>

Neuroendocrine tumours in general are dealt with in the ''[[neuroendocrine tumours]]'' article.

==General==
*Rare ~ 1% of pancreatic tumours.<ref>{{Ref GLP|496}}</ref>
*Presentation depends on subtype, e.g. for ''insulinoma'' the typical presentation is hypoglycemia.
*May be part of a syndrome:
**[[MEN 1|Multiple endocrine neoplasia I]].
**[[Von Hippel-Lindau disease]].<ref name=pmid22370733>{{Cite journal | last1 = Charlesworth | first1 = M. | last2 = Verbeke | first2 = CS. | last3 = Falk | first3 = GA. | last4 = Walsh | first4 = M. | last5 = Smith | first5 = AM. | last6 = Morris-Stiff | first6 = G. | title = Pancreatic Lesions in von Hippel-Lindau Disease? A Systematic Review and Meta-synthesis of the Literature. | journal = J Gastrointest Surg | volume = | issue = | pages = | month = Feb | year = 2012 | doi = 10.1007/s11605-012-1847-0 | PMID = 22370733 }}</ref>
**[[Neurofibromatosis type 1]].<ref name=pmid15249710>{{Cite journal | last1 = Alexakis | first1 = N. | last2 = Connor | first2 = S. | last3 = Ghaneh | first3 = P. | last4 = Lombard | first4 = M. | last5 = Smart | first5 = HL. | last6 = Evans | first6 = J. | last7 = Hughes | first7 = M. | last8 = Garvey | first8 = CJ. | last9 = Vora | first9 = J. | title = Hereditary pancreatic endocrine tumours. | journal = Pancreatology | volume = 4 | issue = 5 | pages = 417-33; discussion 434-5 | month = | year = 2004 | doi = 10.1159/000079616 | PMID = 15249710 }}</ref>

===WHO classification of 2017===
Five categories:
*NET G1. Ki67 Index < 3%, Mitotic Index < 2/10HPF
*NET G2. Ki67 Index 3-20%, Mitotic Index 2-20/10HPF
*NET G3. Ki67 Index > 20%, Mitotic Index > 20/10 HPF AND well-differentiated, expressing neuroendocrine differentiation and hormones
*Neuroendocrine carcinoma (NEC G3). Ki 67 Index > 20%, Mitotic Index > 20/10 HPF Poorly differentiated, expressing neuroendocrine differentiation but lacking exocrine markers.
*[[MiNEN]]. mixed endocrine-nonendocrine neoplasm: components of a non-endocrine carcinoma (mostly ductal adenocarcinoma or acinar cell carcinoma) combined with a neuroendocrine neoplasm

===Classification by product===
Based on peptide produced in the pancreatic islets:
#Glucagon from alpha cells ([[glucagonoma]]).
#Insulin from beta cells (insulinoma) - most common ~ 50% of islet cell tumours.
#Somatostatin from D cells ([[somatostatinoma]]).
#Pancreatic polypeptide from PP cells.

Others:
#Vasoactive intestinal peptide (VIPoma).
#Gastrin (gastrinoma).
#*May be seen in ''[[Zollinger-Ellison syndrome]]''.
#**Triad: pancreatic gastrinoma, gastric acid hypersecretion, marked peptic ulcers in the small bowel.<ref name=pmid13259432>{{cite journal |author=Zollinger RM, Ellison EH |title=Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas |journal=Ann. Surg. |volume=142 |issue=4 |pages=709–23; discussion, 724–8 |year=1955 |pmid=13259432|doi=10.1097/00000658-195510000-00015 |PMC = 1465210 |URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210/?page=1 }}</ref>

==Gross==
*Usually in the head of the pancreas - 68% in one series,<ref name=pmid22869477>{{Cite journal | last1 = Oh | first1 = TG. | last2 = Chung | first2 = MJ. | last3 = Park | first3 = JY. | last4 = Bang | first4 = SM. | last5 = Park | first5 = SW. | last6 = Chung | first6 = JB. | last7 = Song | first7 = SY. | title = Prognostic factors and characteristics of pancreatic neuroendocrine tumors: single center experience. | journal = Yonsei Med J | volume = 53 | issue = 5 | pages = 944-51 | month = Sep | year = 2012 | doi = 10.3349/ymj.2012.53.5.944 | PMID = 22869477 | PMC = 3423842}}</ref> and 50% in another series.<ref name=pmid22869477/>
==Microscopic==
Features:
*Nests of cells or cords.
*Stippled chromatin - '''key feature'''.
*Moderate cytoplasm.
*+/-Hyaline globules.

DDx:
*[[Solid pseudopapillary neoplasm]].
*[[Acinar cell carcinoma]].
*[[Invasive ductal carcinoma of the pancreas]].

===Images===
<gallery>
Image: Pancreatic_insulinoma_(1).JPG | Insulinoma. (WC)
Image: Pancreatic_insulinoma_(2).JPG | Insulinoma. (WC)
Image: Pancreatic insulinoma (3) Chromogranin A.JPG | Insulinoma - chromogranin A. (WC)
Image: Pancreatic insulinoma (4) Insulin immuostain.JPG | Insulinoma - insulin IHC. (WC)
</gallery>
<gallery>
Image: Pancreatic neuroendocrine tumour -- very low mag.jpg | Pancreatic NET - very low mag. (WC/Nephron)
Image: Pancreatic neuroendocrine tumour -- low mag.jpg | Pancreatic NET - low mag. (WC/Nephron)
Image: Pancreatic neuroendocrine tumour -- intermed mag.jpg | Pancreatic NET - intermed. mag. (WC/Nephron)
Image: Pancreatic neuroendocrine tumour - alt -- intermed mag.jpg | Pancreatic NET - intermed. (WC/Nephron) mag.
Image: Pancreatic neuroendocrine tumour -- high mag.jpg | Pancreatic NET - high mag. (WC/Nephron)
Image: Pancreatic neuroendocrine tumour -- very high mag.jpg | Pancreatic NET - very high mag. (WC/Nephron)

Image: Pancreatic neuroendocrine tumour - 2 -- intermed mag.jpg | Pancreatic NET - intermed. mag. (WC/Nephron)
Image: Pancreatic neuroendocrine tumour - 2 -- high mag.jpg | Pancreatic NET - high mag. (WC/Nephron)
</gallery>

[[File: PANC NET LG 1 sl 1.png| Low grade pancreatic neuroendocrine tumor]]
[[File: PANC NET LG 1 sl 2.png| Low grade pancreatic neuroendocrine tumor]]
[[File: PANC NET LG 1 sl 3.png| Low grade pancreatic neuroendocrine tumor]]
[[File: PANC NET LG 1 sl 4.png| Low grade pancreatic neuroendocrine tumor]]
[[File: PANC NET LG 1 sl 5.png| Low grade pancreatic neuroendocrine tumor]]

Low grade pancreatic neuroendocrine tumor. A. Stromal trabeculums (black arrows) support the uniform, non-necrotic tumor that has sharp edges (green arrows). B. Modestly variable, rounded nuclei show open chromatin and nucleoli. Individual pyknotic nuclei (black arrows) are insufficient as evidence of necrosis sufficient to increase grade. C. Immunostain. Cytoplasm is diffusely synaptophysin positive. D. Immunostain. Cytoplasm shows stippled chromogranin positivity. E. Immunostain. Only scattered nuclei are Ki67 positive.

www:
*[http://path.upmc.edu/cases/case172/micro.html Islet cell tumour (upmc.edu)].
*[http://path.upmc.edu/cases/case339.html Pancreatic NET with features of SPT (upmc.edu)].
*[http://path.upmc.edu/cases/case501.html Pancreatic NET - another case (upmc.edu)].

==IHC==
*CK19 +ve.
**Considered a poor prognostic factor<ref name=pmid19956064>{{Cite journal | last1 = Jain | first1 = R. | last2 = Fischer | first2 = S. | last3 = Serra | first3 = S. | last4 = Chetty | first4 = R. | title = The use of Cytokeratin 19 (CK19) immunohistochemistry in lesions of the pancreas, gastrointestinal tract, and liver. | journal = Appl Immunohistochem Mol Morphol | volume = 18 | issue = 1 | pages = 9-15 | month = Jan | year = 2010 | doi = 10.1097/PAI.0b013e3181ad36ea | PMID = 19956064 }}</ref> - disputed by an older paper.<ref>{{Cite journal | last1 = La Rosa | first1 = S. | last2 = Rigoli | first2 = E. | last3 = Uccella | first3 = S. | last4 = Novario | first4 = R. | last5 = Capella | first5 = C. | title = Prognostic and biological significance of cytokeratin 19 in pancreatic endocrine tumours. | journal = Histopathology | volume = 50 | issue = 5 | pages = 597-606 | month = Apr | year = 2007 | doi = 10.1111/j.1365-2559.2007.02662.x | PMID = 17394496 }}</ref>
*Chromogranin +ve.
*Synaptophysin +ve.
*CD56 +ve.
*[[PAX8]] +ve (74% of cases<ref name=pmid20890270>{{Cite journal | last1 = Sangoi | first1 = AR. | last2 = Ohgami | first2 = RS. | last3 = Pai | first3 = RK. | last4 = Beck | first4 = AH. | last5 = McKenney | first5 = JK. | last6 = Pai | first6 = RK. | title = PAX8 expression reliably distinguishes pancreatic well-differentiated neuroendocrine tumors from ileal and pulmonary well-differentiated neuroendocrine tumors and pancreatic acinar cell carcinoma. | journal = Mod Pathol | volume = 24 | issue = 3 | pages = 412-24 | month = Mar | year = 2011 | doi = 10.1038/modpathol.2010.176 | PMID = 20890270 }}</ref>).

Functional tumours:
*Insulin.
*Glucagon.
*Somatostatin.
*Gastrin.

A panel:
*Chromogranin, synaptophysin, CD10, PR, beta-catenin, CK7, pankeratin, Ki-67, CK19.

Notes:
*''CK19'' should '''not''' be confused with ''CA19-9''.
*If the tumour is '''not''' functional (clinically) one can forgo the stains for functional tumours.

==Sign out==
<pre>
TAIL OF PANCREAS AND SPLEEN, PARTIAL PANCREATECTOMY AND SPLENECTOMY:
- CYSTIC PANCREATIC NEUROENDOCRINE TUMOUR, pT1, pN0.
-- INTERMEDIATE GRADE (G2).
-- NARROWLY EXCISED.
-- PLEASE SEE TUMOUR SUMMARY.
- UNREMARKABLE SURROUNDING PANCREAS WITH FAT.
- SPLEEN WITHIN NORMAL LIMITS.
- FOUR BENIGN LYMPH NODES.
</pre>

<pre>
TAIL OF PANCREAS AND SPLEEN, PARTIAL PANCREATECTOMY AND SPLENECTOMY:
- CYSTIC PANCREATIC NEUROENDOCRINE TUMOUR, pT1, pN0.
-- LOW GRADE (G1).
-- SURGICAL MARGINS NEGATIVE.
-- PLEASE SEE TUMOUR SUMMARY.
- UNREMARKABLE SURROUNDING PANCREAS WITH FAT.
- SPLEEN WITHIN NORMAL LIMITS.
- FOUR BENIGN LYMPH NODES.
</pre>

===Micro===
The sections show pancreas with a large well-circumscribed tumour that centrally has a large cyst-like cavity. The tumour cells have moderate pale grey cytoplasm and round nuclei with salt and pepper chromatin. The tumour cells are arranged in cords and nests. No cholesterol clefts are readily apparent. No hyaline globules are identified. No papillary structures are apparent.

No necrosis is identified. No degenerative changes are apparent. Mitotic activity is seen focally. There are 2 mitoses per 10 high power fields, were one high power field has an area of 0.2376 mm*mm.

==See also==
*[[Pancreas]].
*[[Neuroendocrine tumours]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Pancreas]]

Trichilemmoma

2019-02-20T03:37:31Z

Brigitte:

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = SkinTumors-P6190341.JPG
| Width =
| Caption = Trichilemmoma. [[H&E stain]].
| Synonyms =
| Micro = superficial dermal lesion contiguous with the epidermis; core of lesion has cuboidal cells with round nuclei, eosinophilic-clear cytoplasm; periphery of lesion surrounded by hyaline band, has peripheral palisading
| Subtypes =
| LMDDx = [[trichilemmal carcinoma]], [[basal cell carcinoma]], [[inverted follicular keratosis]]
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = [[skin]]
| Assdx =
| Syndromes = [[Cowden syndrome]]
| Clinicalhx =
| Signs =
| Symptoms =
| Prevalence = uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = benign
| Other =
| ClinDDx =
| Tx =
}}
'''Trichilemmoma''' is an uncommon [[Dermatologic neoplasms|skin tumour]].

==General==
*Benign neoplasm with features of the pilosebaceous follicular epithelium.<ref>URL: [http://emedicine.medscape.com/article/1059940-overview http://emedicine.medscape.com/article/1059940-overview]. Accessed on: 2 September 2011.</ref>
*Associated with ''[[nevus sebaceous]]''.<ref name=pmid16503928>{{Cite journal | last1 = Baykal | first1 = C. | last2 = Buyukbabani | first2 = N. | last3 = Yazganoglu | first3 = KD. | last4 = Saglik | first4 = E. | title = [Tumors associated with nevus sebaceous]. | journal = J Dtsch Dermatol Ges | volume = 4 | issue = 1 | pages = 28-31 | month = Jan | year = 2006 | doi = 10.1111/j.1610-0387.2006.05855.x | PMID = 16503928 }}</ref>
*Muliple trichilemmomas associated with [[Cowden syndrome]].<ref name=Ref_Derm386>{{Ref Derm|386}}</ref>

==Microscopic==
Features:<ref name=Ref_Derm386>{{Ref Derm|386}}</ref>
*Superficial dermal lesion contiguous with the epidermis:
**Core of lesion:
***Cuboidal cells with round nuclei, eosinophilic-clear cytoplasm.
**Periphery of lesion:
***Surrounded by hyaline band.
***Peripheral palisading.

DDx:
*[[Trichilemmal carcinoma]].
*[[Basal cell carcinoma]].
*[[Inverted follicular keratosis]].

===Images===
*[http://dermimages.med.jhmi.edu/images/trichilemmoma_1_060109.jpg Trichilemmoma (jhmi.edu)].<ref>URL: [http://dermatlas.med.jhmi.edu/derm/indexDisplay.cfm?ImageID=667496720 http://dermatlas.med.jhmi.edu/derm/indexDisplay.cfm?ImageID=667496720]. Accessed on: 2 September 2011.</ref>
*[http://www.flickr.com/photos/40981620@N04/3812019838/in/pool-1185084@N23/ Trichilemmoma - low mag. (flickr.com/Irlam)].
*[http://www.flickr.com/photos/40981620@N04/3812019930/in/pool-1185084@N23/ Trichilemmoma - intermed. mag. (flickr.com/Irlam)].
*[http://www.flickr.com/photos/40981620@N04/3811204517/in/pool-1185084@N23/ Trichilemmoma - high mag. (flickr.com/Irlam)].

==See also==
*[[Dermatologic neoplasms]].
*[[Cowden syndrome]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Dermatologic neoplasms]]

Pancreatoblastoma

2018-07-24T14:59:21Z

Brigitte: /* Image */

'''Pancreatoblastoma''' is a rare malignant tumour of the [[pancreas]] that is predominantly afflicts children.

==General==
*Very rare.
*Tumour of childhood - age of diagnosis ~5 years old.<ref name=pmid22525406>{{Cite journal | last1 = Glick | first1 = RD. | last2 = Pashankar | first2 = FD. | last3 = Pappo | first3 = A. | last4 = Laquaglia | first4 = MP. | title = Management of pancreatoblastoma in children and young adults. | journal = J Pediatr Hematol Oncol | volume = 34 Suppl 2 | issue = | pages = S47-50 | month = May | year = 2012 | doi = 10.1097/MPH.0b013e31824e3839 | PMID = 22525406 }}</ref>
*Prognosis ~80% year survival in children<ref name=pmid21696948>{{Cite journal | last1 = Bien | first1 = E. | last2 = Godzinski | first2 = J. | last3 = Dall'igna | first3 = P. | last4 = Defachelles | first4 = AS. | last5 = Stachowicz-Stencel | first5 = T. | last6 = Orbach | first6 = D. | last7 = Bisogno | first7 = G. | last8 = Cecchetto | first8 = G. | last9 = Warmann | first9 = S. | title = Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). | journal = Eur J Cancer | volume = 47 | issue = 15 | pages = 2347-52 | month = Oct | year = 2011 | doi = 10.1016/j.ejca.2011.05.022 | PMID = 21696948 }}</ref> more aggressive in adults.
*May be seen in adults.<ref name=pmid22572137>{{Cite journal | last1 = Balasundaram | first1 = C. | last2 = Luthra | first2 = M. | last3 = Chavalidthamrong | first3 = D. | last4 = Chow | first4 = J. | last5 = Khan | first5 = H. | last6 = Endres | first6 = PJ. | title = Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. | journal = JOP | volume = 13 | issue = 3 | pages = 301-3 | month = May | year = 2012 | doi = | PMID = 22572137 }}</ref>

Associations:<ref name=pmid17228135/>
*[[Beckwith-Wiedemann syndrome]].
*[[Familial adenomatous polyposis]].

==Microscopic==
Features:<ref name=pmid17228135>{{Cite journal | last1 = Saif | first1 = MW. | title = Pancreatoblastoma. | journal = JOP | volume = 8 | issue = 1 | pages = 55-63 | month = | year = 2007 | doi = | PMID = 17228135 }}</ref><ref name=pmid15943785/>
*Acinar-like structures.
*Squamoid corpuscles.
*Undifferentiated component.

DDx:
*Other [[small round cell tumours]].
===Image===

==IHC==
Features:<ref name=pmid15943785>{{Cite journal | last1 = Nishimata | first1 = S. | last2 = Kato | first2 = K. | last3 = Tanaka | first3 = M. | last4 = Ijiri | first4 = R. | last5 = Toyoda | first5 = Y. | last6 = Kigasawa | first6 = H. | last7 = Ohama | first7 = Y. | last8 = Nakatani | first8 = Y. | last9 = Notohara | first9 = K. | title = Expression pattern of keratin subclasses in pancreatoblastoma with special emphasis on squamoid corpuscles. | journal = Pathol Int | volume = 55 | issue = 6 | pages = 297-302 | month = Jun | year = 2005 | doi = 10.1111/j.1440-1827.2005.01829.x | PMID = 15943785 }}</ref>
*[[CK7]] +ve - acinar, undifferentiated component.
*CK8 +ve - squamous component.
*CK18 +ve - squamous component.
*[[CK19]] +ve - squamous component.

==See also==
*[[Small round cell tumours]].
*[[Pancreas]].

==References==
{{Reflist|1}}

[[Category:Gastrointestinal pathology]]
[[Category:Diagnosis]]

Graft-versus-host disease

2018-06-20T13:50:58Z

Brigitte: /* Microscopic (intestine) */

'''Graft-versus-host disease''', abbreviated as '''GVHD''', is a rare thingy seen mostly in tertiary care centres. It is a complication of [[hematopoietic stem cell transplantation]].

==General==
*Complication of hematopoietic stem cell transplantation, i.e. bone marrow transplantation (BMT).
**Affects skin, liver (bile ducts), gastrointestinal tract.<ref name=pmid15792120>{{cite journal |author=Niino D, Nakashima M, Kondo H, ''et al.'' |title=Correlation of donor-derived keratinocytes and severity of graft-versus-host disease (GVHD) in epidermis |journal=Pathol. Res. Pract. |volume=200 |issue=11-12 |pages=775–81 |year=2005 |pmid=15792120 |doi= |url=}}</ref><ref>{{cite journal |author=van Dijk AM, Kessler FL, Verdonck LF, ''et al.'' |title=Primary human keratinocytes as targets in predicting acute graft-versus-host disease following HLA-identical bone marrow transplantation |journal=Br. J. Haematol. |volume=111 |issue=3 |pages=791–6 |year=2000 |month=December |pmid=11122139 |doi= |url=}}</ref>
*The histology of GVHD in the intestine is the same as rejection in bowel transplantation.<ref>GT. 14 January 2011.</ref>

Clinical:
*May present as diarrhea.
*Main DDx (clinical): infection.

==Microscopic==
===Microscopic (skin)===
Features:<ref name=stanford>{{cite web |url=http://surgpathcriteria.stanford.edu/transplant/skinacutegvhd/printable.html |title=Acute Graft versus Host Disease of the Skin |last1= |first1= |last2= |first2= |date= |work= |publisher= |accessdate=January 17, 2011}}</ref>
#Keratinocyte [[apoptosis]].
#*Intensely eosinophilic on [[H&E]].
#Epidermotropic lymphocytic infiltrate = lymphocytes in the epidermis.
#Vacuolar degeneration of basal and suprabasal cells in the epidermis.

Note:
*Apoptotic cells should not be confused with dyskeratotic cells.<ref>{{cite web |url=http://dermatology.acvsc.org.au/dermatology_assets/documents/proc2007/acvs%20dermatology%20chapter%20proceedings%202007%20-%20nimmo%20-%20dyskeratotic,%20apoptotic%20or%20acantholytic%20keratinocytes.pdf |title=Dyskeratotic, apoptotic or acantholytic keratinocytes? How to differentiate these on histology and what meaning does this have to the disease in question |author=Judith S. Nimmo |date= |work= |publisher= |accessdate=17 January 2011}}</ref>

====Grading<ref name=stanford/>====
*Grade I: Only vacuolar changes, no apoptosis, no lymphocytes; '''not treated'''.
*Grade II: Only scattered apoptotic cells.
*Grade III: Focal separation/cleft formation at the dermal-epidermal junction.
*Grade IV: Extensive necrosis with degeneration of epidermis.

Notes:
*Same scheme applies to esophagus... it has the same structure.
*Originally described in NEJM.<ref name=pmid235092>{{cite journal |author=Thomas ED, Storb R, Clift RA, ''et al.'' |title=Bone-marrow transplantation (second of two parts) |journal=N. Engl. J. Med. |volume=292 |issue=17 |pages=895–902 |year=1975 |month=April |pmid=235092 |doi=10.1056/NEJM197504242921706 |url=}}</ref>

===Microscopic (intestine)===
Features:<ref name=pmid20953169>{{cite journal |author=Cogbill CH, Drobyski WR, Komorowski RA |title=Gastrointestinal pathology of autologous graft-versus-host disease following hematopoietic stem cell transplantation: a clinicopathological study of 17 cases |journal=Mod. Pathol. |volume=24 |issue=1 |pages=117–25 |year=2011 |month=January |pmid=20953169 |doi=10.1038/modpathol.2010.163 |url=http://www.nature.com/modpathol/journal/v24/n1/full/modpathol2010163a.htm}}</ref>
*Isolated epithelial cell apoptosis - '''key feature'''.
*+/-Crypt destruction (focal or extensive).
*+/-Loss of epithelium (denudation).

Notes:
*Neutrophils should not be present.

Images:

====Grading<ref name=pmid20953169/>====
*Grade 1 = isolated epithelial cell apoptosis.
**No crypt loss/destruction.
*Grade 2 = individual crypts are lost/scatter destruction of single crypts.
*Grade 3 = foci several adjacent crypts lost.
*Grade 4 = large number of adjacent crypts lost/loss of epithelium.

Notes:
*Low-grade rejection is a diagnosis that requires a careful examination, i.e. it is subtle.

===Microscopic (liver)===
:See: ''[[Vanishing bile duct syndrome]]''.

==See also==
*[[Hematopoietic stem cell transplantation]].
*[[Small bowel]].
*[[Colon]].
*[[Gastrointestinal pathology]].

==References==
{{Reflist|2}}

[[Category:Gastrointestinal pathology]]
[[Category:Haematopathology]]

Gestational trophoblastic disease

2018-05-10T15:11:04Z

Brigitte: /* Gross */

'''Gestational trophoblastic disease''' (abbreviated '''GTD'''), also '''gestational trophoblastic neoplasia''' (abbreviated '''GTN'''), includes [[choriocarcinoma]] and hydatidiform moles.

=Overview=
===Most common===
Overview of gestational trophoblastic disease:
{| class="wikitable sortable"
! Type of mole
! Gross
! [[Nuclear atypia]]
! [[Chorionic villi]]
! [[IHC]]
! DNA content
! Micrographs
|-
| Complete mole
| "snowstorm"
| +/- ?
| yes, all abnormal
| p57(KIP2) -ve
| Paternal, diploid
| [http://commons.wikimedia.org/wiki/File:Intermediate_trophoblast_3_-_low_mag.jpg complete mole + intermed. trophoblast (WC)], [http://en.wikipedia.org/wiki/File:Hydatidiform_mole_%281%29_complete_type.jpg complete mole (WC)]
|-
| Partial mole
| grape-like<br>clusters
| +/-
| large villi, villi with cisterns, <br>villi with cytotrophoblastic inclusions
| p57(KIP2) +ve
| Maternal & paternal, tripoid
| [http://library.med.utah.edu/WebPath/PLACHTML/PLAC064.html partial mole (utah.edu)]
|-
| Choriocarcinoma
| haemorrahagic, necrotic
| marked
| none
| beta-hCG +ve
| ?
| [[Image:Choriocarcinoma_-2-_very_high_mag.jpg|thumb|center|120px|Choriocarcinoma. (WC)]]
|-
|}

===More comprehensive overview===
Benign abnormal looking placenta:
*[[Placental site nodule]] (PSN).
*[[Exaggerated placental site]] (EPS).

Abnormal fertilization:
*[[Hydatidiform mole]].

Tumours:
*Invasive mole.
*[[Choriocarcinoma]].
*[[Placental site trophoblastic tumour]] (PSTT).
*[[Epithelioid trophoblastic tumour]] (ETT).

=Entities=
==Choriocarcinoma==
{{Main|Choriocarcinoma}}

==Hydatidiform moles==
===General===
*Significance: increased risk for choriocarcinoma (in complete moles).
*Non-neoplastic proliferation.

Etymology:
*''Hydatid'' is literally ''watery vesicle''.<ref>URL: [http://dictionary.reference.com/browse/hydatid http://dictionary.reference.com/browse/hydatid].</ref>

====Types====
#Partial mole - see [[partial mole]].
#Complete mole - see [[complete mole]].

Extent:
*Invasive mole - '''not''' a subtype.
**Within uterine muscle +/- vessels.

===Microscopic===
Hydropic changes:
{| class="wikitable sortable"
! Entity
! [[Chorionic villi]] (outline)
! Cisterns
! [[Blood vessel]]s
! Nucleated RBCs
! p57 / Ki-67<ref>URL: [http://www.ihcworld.com/_newsletter/2003/focus_mar_2003.pdf http://www.ihcworld.com/_newsletter/2003/focus_mar_2003.pdf]. Accessed on: 28 May 2011.</ref> staining ‡
! Ploidy
! Micrograph
|-
| Complete mole
| bizarre; often not ovoid; fissures/slit-like gaps
| well-developed
| canalicular (thin walled) / few (???)
| rare
| -ve / ~70%
| diploid / tetraploid
| [http://commons.wikimedia.org/wiki/File:Hydatidiform_mole_%281%29_complete_type.jpg], [http://commons.wikimedia.org/wiki/File:Hydatidiform_mole_%282%29_complete_type.jpg], [http://commons.wikimedia.org/wiki/File:Hydatidiform_mole_%283%29_complete_type.jpg], [http://commons.wikimedia.org/wiki/File:Hydatidiform_mole_%284%29_complete_type.jpg]
|-
| Partial mole
| jagged, still quasi ovoid
| poorly developed / small
| present
| common
| +ve / ~70%
| triploid
| [http://www.flickr.com/photos/78147607@N00/571279403], [http://www.flickr.com/photos/78147607@N00/570796738]
|-
| Hydropic abortus
| smooth
| poorly developed / small
| common
| common
| +ve / ~20%
| diploid
| [http://www.ipath-network.com/ipath/object/view/181344]
|}
Note:
* ‡ The amount of [[Ki-67]] staining varies considerably based on what one reads. Chen ''at al.''<ref>{{Cite journal | last1 = Chen | first1 = Y. | last2 = Shen | first2 = D. | last3 = Gu | first3 = Y. | last4 = Zhong | first4 = P. | last5 = Xie | first5 = J. | last6 = Song | first6 = Q. | title = The diagnostic value of Ki-67, P53 and P63 in distinguishing partial Hydatidiform mole from hydropic abortion. | journal = Wien Klin Wochenschr | volume = 124 | issue = 5-6 | pages = 184-7 | month = Mar | year = 2012 | doi = 10.1007/s00508-011-0119-4 | PMID = 22218717 }}</ref> suggest 25% versus 5% for partial mole versus hydropic abortus.

====Mole versus normal====
*Moles have large [[chorionic villi]] with edema and abnormal blood vessels.<ref>URL: [http://pathologyoutlines.com/placenta.html#hydatgeneral http://pathologyoutlines.com/placenta.html#hydatgeneral].</ref>

====Non-molar versus partial versus complete - short version====
Features:<ref name=pmid8157742>{{Cite journal | last1 = Howat | first1 = AJ. | last2 = Beck | first2 = S. | last3 = Fox | first3 = H. | last4 = Harris | first4 = SC. | last5 = Hill | first5 = AS. | last6 = Nicholson | first6 = CM. | last7 = Williams | first7 = RA. | title = Can histopathologists reliably diagnose molar pregnancy? | journal = J Clin Pathol | volume = 46 | issue = 7 | pages = 599-602 | month = Jul | year = 1993 | doi = | PMID = 8157742 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC501384/?page=3 }}</ref>
*Non-molar pregnancy: polar proliferation of trophoblastic tissue.
*Partial mole: Norwegian fjord periphery, circumferential or multifocal trophoblastic proliferation, fetal parts.
*Complete mole: grapes grossly, large villi with round borders.

===IHC===
*p57(KIP2) - the gene is strongly paternally imprinted and the paternal copy is inactived; its expression is from the maternal gene.
**Complete moles lack the maternal genome; thus, p57(KIP2) immunostaining (in the cytotrophoblasts and villous stromal cells) is absent.<ref name=pmid15754295>{{cite journal |author=Merchant SH, Amin MB, Viswanatha DS, Malhotra RK, Moehlenkamp C, Joste NE |title=p57KIP2 immunohistochemistry in early molar pregnancies: emphasis on its complementary role in the differential diagnosis of hydropic abortuses |journal=Hum. Pathol. |volume=36 |issue=2 |pages=180–6 |year=2005 |month=February |pmid=15754295 |doi=10.1016/j.humpath.2004.12.007 |url=}}</ref><ref name=pmid12514787>{{Cite journal | last1 = Fukunaga | first1 = M. | title = Immunohistochemical characterization of p57(KIP2) expression in early hydatidiform moles. | journal = Hum Pathol | volume = 33 | issue = 12 | pages = 1188-92 | month = Dec | year = 2002 | doi = 10.1053/hupa.2002.129421 | PMID = 12514787 }}</ref>
***Intermediate trophoblasts and maternal tissue are positive controls.<ref name=pmid12514787/>
**Memory device:
***'''''p'''57 is '''p'''ositive in '''p'''artial moles''.
***'''3 Ps''' - partial moles are triploid.

===Molecular===
*The type of mole can be determined by [[cytogenetics]].<ref>[http://jcp.bmjjournals.com/cgi/reprint/51/6/438.pdf http://jcp.bmjjournals.com/cgi/reprint/51/6/438.pdf]</ref>

==Partial hydatidiform mole==
*[[AKA]] ''partial mole''.

===General===
Genetics:
*Usually triploid (e.g. 69XXY).

===Microscopic===
Features:
*Abnormal chorionic villi.
**Villi too large (>0.1 mm ?).
**Villi with cisterns.
***Contain fluid in the centre, i.e. are "hydropic".
**Villi with cytotrophoblastic inclusions.
***[[Cytotrophoblast]] in the core of a villus (normally it is only at the surface of the villus).
*May have fetal parts, such as nucleated RBCs.
*Trophoblastic proliferation.
**Without atypia.<ref>URL: [http://pathologyoutlines.com/placenta.html#incompletemole http://pathologyoutlines.com/placenta.html#incompletemole]. Accessed on: 9 August 2011.</ref>
*"Norwegian fjord periphery"<ref name=pmid8157742>{{Cite journal | last1 = Howat | first1 = AJ. | last2 = Beck | first2 = S. | last3 = Fox | first3 = H. | last4 = Harris | first4 = SC. | last5 = Hill | first5 = AS. | last6 = Nicholson | first6 = CM. | last7 = Williams | first7 = RA. | title = Can histopathologists reliably diagnose molar pregnancy? | journal = J Clin Pathol | volume = 46 | issue = 7 | pages = 599-602 | month = Jul | year = 1993 | doi = | PMID = 8157742 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC501384/?page=3 }}</ref> - jagged border / irregular sawtooth-like periphery.
**Complete moles tend to have a smooth border

DDx:
*[[Complete hydatidiform mole]].
*[[Placental mesenchymal dysplasia]].
*Hydropic abortus - see [[products of conception]] and [[chorionic villi]].

Images:
*[http://www-medlib.med.utah.edu/WebPath/PLACHTML/PLAC067.html Partial mole (med.utah.edu)].
*[http://www.gfmer.ch/selected_images_v2/detail_list.php?cat1=12&cat2=86&cat3=795&cat4=3&stype=n Partial mole - several images (gfmer.ch)].

===IHC===
Features:<ref name=pmid22218717>{{Cite journal | last1 = Chen | first1 = Y. | last2 = Shen | first2 = D. | last3 = Gu | first3 = Y. | last4 = Zhong | first4 = P. | last5 = Xie | first5 = J. | last6 = Song | first6 = Q. | title = The diagnostic value of Ki-67, P53 and P63 in distinguishing partial Hydatidiform mole from hydropic abortion. | journal = Wien Klin Wochenschr | volume = 124 | issue = 5-6 | pages = 184-7 | month = Mar | year = 2012 | doi = 10.1007/s00508-011-0119-4 | PMID = 22218717 }}</ref>
*Ki-67 ~ 25+/-5% of cytotrophoblasts and intermediate trophoblasts.
**Hydropic abortus ~ 5+/-1%.
*p53 ~ 22+/-12% of cytotrophoblasts and intermediate trophoblasts.
**Hydropic abortus ~ 5+/-3%.

==Complete hydatidiform mole==
*[[AKA]] ''complete mole'', [[AKA]] ''classic mole''.

===General===
Epidemiology:
*May precede [[choriocarcinoma]]<ref name=Ref_PBoD1111>{{Ref PBoD|1111}}</ref> ~ 1-2% risk.

Genetics:
*Diploid - most are 46XX.
*Male derived, i.e. arise from DNA in sperm; empty egg fertilized.

===Gross/Radiology===
*"Snowstorm" appearance on ultrasound.<ref>URL:[http://www.jultrasoundmed.org/cgi/content/abstract/18/9/589 http://www.jultrasoundmed.org/cgi/content/abstract/18/9/589]. Accessed on: 27 July 2010.</ref>
*May be described as "grape-like" on gross exam.<ref name=pmid18162339>{{Cite journal | last1 = Abike | first1 = F. | last2 = Temizkan | first2 = O. | last3 = Payasli | first3 = A. | last4 = Avsar | first4 = F. | last5 = Karahan | first5 = N. | last6 = Baspinar | first6 = S. | title = Postmenopausal complete hydatidiform mole: a case report. | journal = Maturitas | volume = 59 | issue = 1 | pages = 95-8 | month = Jan | year = 2008 | doi = 10.1016/j.maturitas.2007.10.005 | PMID = 18162339 }}</ref>

Image:
*[http://library.med.utah.edu/WebPath/PLACHTML/PLAC063.html Complete mole (utah.edu)].

===Microscopic===
Features:
*No normal villi.
*No fetal parts seen.
**Very rarely nucleated [[RBC]]s.

====Images====
<gallery>
Image:Intermediate_trophoblast_3_-_low_mag.jpg | Complete mole and intermediate trophoblast - intermed. mag. (WC)
Image:Hydatidiform_mole_%281%29_complete_type.jpg | Complete mole - low mag. (WC)
Image:Hydatidiform_mole_%282%29_complete_type.jpg | Complete mole - high mag. (WC)
</gallery>

==Invasive hydatidiform mole==
*[[AKA]] ''invasive mole''.
*[[AKA]] ''chorioadenoma destruens''.<ref name=pmid6300738>{{Cite journal | last1 = McDonald | first1 = TW. | last2 = Ruffolo | first2 = EH. | title = Modern management of gestational trophoblastic disease. | journal = Obstet Gynecol Surv | volume = 38 | issue = 2 | pages = 67-83 | month = Feb | year = 1983 | doi = | PMID = 6300738 }}</ref>
===General===
*This is not a distinct subtype - see ''[[hydatidiform mole]]''.

===Microscopic===
Features:
*[[Chorionic villi]] - abnormal +/- normal.
*Trophoblastic cells within uterine muscle +/- vessels - '''key feature'''.

DDx:
*[[Choriocarcinoma]] - lack [[chorionic villi]], usu. hemorrhagic.

====Images====
<gallery>
Image:Invasive hydatidiform mole - very low mag.jpg | Invasive mole - very low mag. (WC)
Image:Invasive_hydatidiform_mole_-_low_mag.jpg | Invasive mole - low mag. (WC)
Image:Invasive_hydatidiform_mole_-_intermed_mag.jpg | Invasive mole - intermed. mag. (WC)
Image:Invasive_hydatidiform_mole_-_high_mag.jpg | Invasive mole - high mag. (WC)
Image:Invasive hydatidiform mole - very high mag.jpg | Invasive mole - very high mag. (WC)
</gallery>

=Entities - intermediate trophoblast=
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Entity
! Key feature
! Other histologic features
! IHC
! DDx
! Other
! Image
|-
| [[Placental site nodule]] (PSN)
| paucicellular, hyaline material
| no mitotic activity
| p16 -ve, MIB1 low
| [[EPS]], [[squamous carcinoma]]
| post-partum
| [[Image:Placental_site_nodule_-_intermed_mag.jpg |thumb| center| 80px|PSN. (WC)]] [http://www.ijpmonline.org/viewimage.asp?img=IndianJPatholMicrobiol_2009_52_2_240_48931_u4.jpg (ijpmonline.org)]
|-
| [[Exaggerated placental site]] (EPS)
| abundant [[intermediate trophoblast]]s - between muscle
| no mitotic activity
| MIB1 ~0%
| [[PSTT]], [[PSN]]
| post-partum
| Image?
|-
| [[Placental site trophoblastic tumour]] (PSTT)
| abundant cytoplasm - not clear, dyscohesive
| +/-multinucleation
| MIB1 high, p63 -ve, CD146 +ve
| [[EPS]], [[choriocarcinoma]]
| Other?
| [http://www.webpathology.com/case.asp?case=588 (webpathology.com)]
|-
| [[Epithelioid trophoblastic tumour]] (ETT)
| nests of cells in hyaline stroma
| eosinophilic cytoplasm, central nucleus
| MIB1 low, p63 +ve, CD146 -ve
| [[squamous carcinoma]]
| Other?
| [http://www.webpathology.com/image.asp?case=589&n=2 (webpathology.com)]
|-
| [[Choriocarcinoma]]
| polygonal cells with clear cytoplasm ([[cytotrophoblast]]s)
| multinucleated cells with smudged nuclei ([[syncytiotrophoblast]]s), '''no''' [[chorionic villi]]
| beta-hCG +ve, p63 +ve
| [[invasive hydatidiform mole]], [[PSTT]]
| elevated beta-hCG (serum); '''not''' intermediate trophoblast derived.
| [[Image:Choriocarcinoma_-2-_very_high_mag.jpg|thumb|center|120px|Choriocarcinoma. (WC)]] [http://www.webpathology.com/image.asp?case=36&n=1 (webpathology.com)]
|- 
|}

==Placental site nodule==
*Abbreviated ''PSN''.
{{Main|Placental site nodule}}

==Exaggerated placental site==
*Abbreviated ''EPS''.
*Previously known as ''syncytial endometritis''.<ref>URL: [http://www.webpathology.com/image.asp?case=565&n=7 http://www.webpathology.com/image.asp?case=565&n=7]. Accessed on: 22 May 2014.</ref>
{{Main|Exaggerated placental site}}

==Placental site trophoblastic tumour==
*Abbreviated ''PSTT''.
*Malignant counterpart of ''exaggerated placental site'' (abbreviated ''EPS'').
===General===
*Derived from ''intermediate trophoblast''.
*Follows pregnancy.
*May be associated with [[nephrotic syndrome]]<ref name=pmid15457847>{{Cite journal | last1 = Bonazzi | first1 = C. | last2 = Urso | first2 = M. | last3 = Dell'Anna | first3 = T. | last4 = Sacco | first4 = S. | last5 = Buda | first5 = A. | last6 = Cantú | first6 = MG. | title = Placental site trophoblastic tumor: an overview. | journal = J Reprod Med | volume = 49 | issue = 8 | pages = 585-8 | month = Aug | year = 2004 | doi = | PMID = 15457847 }}</ref> with granular IgM staining.<ref>{{Cite journal | last1 = Komatsuda | first1 = A. | last2 = Nakamoto | first2 = Y. | last3 = Asakura | first3 = K. | last4 = Yasuda | first4 = T. | last5 = Imai | first5 = H. | last6 = Miura | first6 = AB. | title = Case report: nephrotic syndrome associated with a total hydatidiform mole. | journal = Am J Med Sci | volume = 303 | issue = 5 | pages = 309-12 | month = May | year = 1992 | doi = | PMID = 1580319 }}</ref>

Clinical:
*Raised (serum) beta-hCG - but usually not has high as in [[choriocarcinoma]].
**In ~70% < 1000 IU/L.<ref name=pmid19552948>{{Cite journal | last1 = Schmid | first1 = P. | last2 = Nagai | first2 = Y. | last3 = Agarwal | first3 = R. | last4 = Hancock | first4 = B. | last5 = Savage | first5 = PM. | last6 = Sebire | first6 = NJ. | last7 = Lindsay | first7 = I. | last8 = Wells | first8 = M. | last9 = Fisher | first9 = RA. | title = Prognostic markers and long-term outcome of placental-site trophoblastic tumours: a retrospective observational study. | journal = Lancet | volume = 374 | issue = 9683 | pages = 48-55 | month = Jul | year = 2009 | doi = 10.1016/S0140-6736(09)60618-8 | PMID = 19552948 }}</ref>
**In a series of 55 cases the average beta-hCG was ~700 IU/L.<ref name=pmid16246400>{{Cite journal | last1 = Baergen | first1 = RN. | last2 = Rutgers | first2 = JL. | last3 = Young | first3 = RH. | last4 = Osann | first4 = K. | last5 = Scully | first5 = RE. | title = Placental site trophoblastic tumor: A study of 55 cases and review of the literature emphasizing factors of prognostic significance. | journal = Gynecol Oncol | volume = 100 | issue = 3 | pages = 511-20 | month = Mar | year = 2006 | doi = 10.1016/j.ygyno.2005.08.058 | PMID = 16246400 }}</ref>
*Prognosis dependent on time of diagnosis from last pregnancy.
**<48 months = good prognosis.<ref name=pmid19552948/>

===Microscopic===
Features:
*Large cells:
**Nuclear pleomorphism.
**Cytoplasm:
***Abundant.
***Solid, i.e. not vesicular.
***Light basophilic, not clear - '''key feature'''.
**[[NC ratio]] ~ normal.
*+/-Multinucleated cells.
*Ectatic blood vessels.

Note:
*'''No''' chorionic villi.
**If villi are present... it is probably a [[hydatidiform mole]].

DDx:
*[[Exaggerated placental site]] - EPS has near zero Ki-67.
*[[Choriocarcinoma]] - choriocarcinoma biphasic.<ref>URL: [http://www.webpathology.com/image.asp?n=3&Case=588 http://www.webpathology.com/image.asp?n=3&Case=588]. Accessed on: 1 January 2012.</ref>

Images:
*[http://www.webpathology.com/case.asp?case=588 Collection of PSTT images (webpathology.com)].

===IHC===
Features:<ref>{{Cite journal | last1 = Shih | first1 = IM. | last2 = Kurman | first2 = RJ. | title = Ki-67 labeling index in the differential diagnosis of exaggerated placental site, placental site trophoblastic tumor, and choriocarcinoma: a double immunohistochemical staining technique using Ki-67 and Mel-CAM antibodies. | journal = Hum Pathol | volume = 29 | issue = 1 | pages = 27-33 | month = Jan | year = 1998 | doi = | PMID = 9445130 }}</ref>
*CD146 +ve.
*p63 -ve.
*Ki-67 ~14+/-7%.
**Choriocarcinoma ~69+/-20%.

==Epithelioid trophoblastic tumour==
*Abbreviated ''ETT''.
===General===
*Often in endocervix.
*Malignant counterpart of ''placental site nodule'' or ''PSN''.

Clinical:
*Vaginal bleeding.
*Elevated beta-hCG.

===Gross===
Features:<ref name=pmid16258513>{{Cite journal | last1 = Fadare | first1 = O. | last2 = Parkash | first2 = V. | last3 = Carcangiu | first3 = ML. | last4 = Hui | first4 = P. | title = Epithelioid trophoblastic tumor: clinicopathological features with an emphasis on uterine cervical involvement. | journal = Mod Pathol | volume = 19 | issue = 1 | pages = 75-82 | month = Jan | year = 2006 | doi = 10.1038/modpathol.3800485 | PMID = 16258513 }}</ref>
*Solid mass.
*Flesh-like appearance.

Image:

===Microscopic===
Features:<ref name=webp_ett>URL: [http://www.webpathology.com/image.asp?case=589&n=2 http://www.webpathology.com/image.asp?case=589&n=2]. Accessed on: 15 August 2011.</ref>
*Architecture: nests in hyaline matrix.
*Cytoplasm: abundant, eosinophilic.

DDx:
*Invasive [[squamous cell carcinoma]].

Images:
*[http://www.webpathology.com/image.asp?case=589&n=2 ETT (webpathology.com)].<ref name=webp_ett/>
*[http://www.gfmer.ch/selected_images_v2/detail_list.php?cat1=12&cat3=861&stype=d ETT - several images (gfmer.ch)].

===IHC===
Features:<ref name=pmid16931955>{{Cite journal | last1 = Mao | first1 = TL. | last2 = Seidman | first2 = JD. | last3 = Kurman | first3 = RJ. | last4 = Shih | first4 = IeM. | title = Cyclin E and p16 immunoreactivity in epithelioid trophoblastic tumor--an aid in differential diagnosis. | journal = Am J Surg Pathol | volume = 30 | issue = 9 | pages = 1105-10 | month = Sep | year = 2006 | doi = 10.1097/01.pas.0000209854.28282.87 | PMID = 16931955 }}</ref>
*Cyclin E +ve (nuclear).
*p16 -ve.
**+ve (nuclear) in squamous cell carcinoma of the cervix.

Others:
*HMCK -ve.
**SCC +ve.

Note:
*p63 not useful... +ve in both SCC and ETT.

=See also=
*[[Hydatid disease]] - due to Echinoccus spp. such as E. granulosus.
*[[Chorionic villi]].
*[[Ectopic pregnancy]].
*[[Placenta]].
*[[Arias-Stella reaction]] - benign atypical changes of the endometrium associated with trophoblastic tissue.

=References=
{{reflist|2}}

[[Category:Gynecologic pathology]]

Placenta

2018-05-10T15:03:07Z

Brigitte: /* Cord hematoma */

[[Image:Human_placenta.jpg|thumb|right|A placenta (fetal aspect) with attached umbilical cord. (WC/Asturnut)]]
The '''placenta''' feeds the developing baby, breathes for it and disposes of its waste.

The organ is one that seems to be left behind; at least one review suggests it isn't done so well by general pathologists.<ref name=pmid12033960>{{Cite journal | last1 = Sun | first1 = CC. | last2 = Revell | first2 = VO. | last3 = Belli | first3 = AJ. | last4 = Viscardi | first4 = RM. | title = Discrepancy in pathologic diagnosis of placental lesions. | journal = Arch Pathol Lab Med | volume = 126 | issue = 6 | pages = 706-9 | month = Jun | year = 2002 | doi = 10.1043/0003-9985(2002)1260706:DIPDOP2.0.CO;2 | PMID = 12033960 }}</ref>

''Placental pathology'' redirects to this article.

=Clinical=
==Examination of the placenta==
*Most placentas are ''not'' examined by a pathologist.

===Indications for exam by pathology===
Some indications for exam by a pathologist:
*Abnormalities in the:
*#Fetus:
*#*Bad fetal outcome.
*#*Suspected or known congenital abnormalities ''or'' chromosomal abnormalities.
*#*[[IUGR]].
*#Mother:
*#*Infection/suspected infection.
*#*Pre-term labour.
*#*Maternal disease (e.g. [[SLE]], coagulopathy).
*#*Complicated pregnancy (preclampsia, pregnancy induced hypertension, gestational diabetes).
*#Placenta:
*#*Unusual gross characteristics.<ref name=pmid9518951>{{cite journal |author=Yetter JF |title=Examination of the placenta |journal=Am Fam Physician |volume=57 |issue=5 |pages=1045–54 |year=1998 |month=March |pmid=9518951 |doi= |url=}}</ref>

A more detailed list is given by Hargitai et al.<ref name=pmid15280396>{{cite journal |author=Hargitai B, Marton T, Cox PM |title=Best practice no 178. Examination of the human placenta |journal=J. Clin. Pathol. |volume=57 |issue=8 |pages=785–92 |year=2004 |month=August |pmid=15280396 |pmc=1770400 |doi=10.1136/jcp.2003.014217 |url=}}</ref> and Chang.<ref>URL: [http://smj.sma.org.sg/5012/5012ra1.pdf http://smj.sma.org.sg/5012/5012ra1.pdf]. Accessed on: 11 February 2011.</ref>

====Most common====
Most common reasons for submitting a placenta to pathology:<ref>Sherman C. 8 February 2011.</ref>
# Prematurity.
# PROM / possible [[chorioamnionitis]].
# Multiple gestation.

==Bleeding in late pregnancy==
DDx of bleeding in late pregnancy:
*[[Placental abruption]] (most common).
*Placenta previa.
*Vasa previa (fetus losing blood).

==Clinical screening tests==
{{main|Pregnancy}}
*PAPP-A - low values seen in aneuploidy.<ref>URL: [http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=5069 http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=5069]. Accessed on: 7 July 2010.</ref>

==Abbreviations==
*C/S = Caesarean section.
*LSCS = lower segment C-section.
*FTP = failure to progress.
*PROM = premature rupture of membranes.
*PPROM = preterm premature ruptures of membranes.
*IUGR = [[intrauterine growth restriction]].
*IOL = induction of labour.

=Normal histology=
==Villi==
{{Main|Chorionic villi}}
This is dealt with in a separate article that also covers the types of trophoblast ([[cytotrophoblast]], [[syncytiotrophoblast]], intermediate trophoblast).

==Cord==
===Omphalomesenteric duct remnant===
*[[AKA]] vitelline duct.
*Benign embryologic remnant.

Features:
*Duct with benign looking cuboidal epithelium.

===Allantoic duct remnant===
*Benign embryologic remnant.

Features:
*Duct with benign looking flat epithelium.

===Vitelline artery remnant===
Features:
*Small artery in the cord.

==Membranes==
Fetus to mother:
*Amnion - thin layer: one cell layer, basement membrane, connective tissue.
*Cleft - artifactual - empty space.
*Chorion - vascular.
*Decidua (maternal tissue) - may contain obsolete chorionic villi; place to look for hypertensive changes.

===Amnion===
General:
*Next to fetus, surrounds amniotic fluid, avascular.

Characteristics:
*Characterized by a single layer of cells.<ref name=Ref_H4P2_974>{{Ref H4P2|974}}</ref>
**Cuboidal/squamoid shape.
**Eosinophilic cytoplasm.
**Central nucleus.
*Squamous metaplasia may be seen at cord insertion.
*Basement membrane.
*'Compact layer'.<ref name=Ref_H4P2_974>{{Ref H4P2|974}}</ref>
*'Fibroblastic layer'.<ref name=Ref_H4P2_974>{{Ref H4P2|974}}</ref>

===Chorion===
General:
*Surrounds amnion.

Characteristics:
*Layers:<ref name=Ref_H4P2_977>{{Ref H4P2|977}}</ref>
**'Reticular layer' - cellular (inner aspect).
**'Pseudo-basemement membrane'.
**'Outer trophoblastic layer'.
*Has blood vessels.
*Opposed to "trophoblastic X cells" on side opposite of amnion.<ref name=Ref_H4P2_974>{{Ref H4P2|974}}</ref>
**Beneath of the "trophoblastic X cells" is ''decidua'' (mnemonic ''NEW'' = nucleus central, eosinophilic, well-defined cell border), which is maternal tissue.

Note:
*Fibrin deposition may be found deep to the chorion - known as ''subchorionic fibrin deposition''.
**Gross: subchorionic, white/yellow, laminated, classically has a triangular-shape with the base of triangle parallel to fetal aspect of disc.
***Arises due to localized stasis of the inter-villous maternal blood.
**Focal small deposits are considered to be a normal finding - seen in ~15% of cases.<ref name=pmid21393870>{{Cite journal | last1 = Narasimha | first1 = A. | last2 = Vasudeva | first2 = DS. | title = Spectrum of changes in placenta in toxemia of pregnancy. | journal = Indian J Pathol Microbiol | volume = 54 | issue = 1 | pages = 15-20 | month = | year = | doi = 10.4103/0377-4929.77317 | PMID = 21393870 |URL = http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2011;volume=54;issue=1;spage=15;epage=20;aulast=Narasimha }}</ref><ref>URL: [http://zulekhahospitals.com/uploads/files/Sub-chorionic.pdf http://zulekhahospitals.com/uploads/files/Sub-chorionic.pdf]. Accessed on: 17 August 2012.</ref>
***The pathologic counterpart of this is ''[[perivillous fibrin deposition]]''.

Image:
*[http://www.ijpmonline.org/viewimage.asp?img=IndianJPatholMicrobiol_2011_54_1_15_77317_u5.jpg Subchorionic fibrin deposition (ijpmonline.org)].

==Common terms==
*Chorionic plate - fetal aspect of placenta.
*Basal plate - maternal aspect of placenta.
**Has extravillous trophoblast.
**Place to look for maternal vessels.

=Grossing=
This is often very quick. The gross is quite important, as some things cannot be diagnosed microscopically.
==General==
*Dimensions:
**Disc.
**Length of cord, diameter of cord.
**Accessory lobes - dimensions.
***Two lobes of equal size + cord arises in between = bilobate placenta.
*Mass (weight).
**Should be done 'trimmed' (cord cut-off, membrane cut-off).
**Should be done when placenta is "fresh", i.e. not fixed -- as mass tables are based on fresh state.
*Umbilical cord
**Attachment.
***Location: central, eccentric, marginal.
****Marginal attachment assoc. with hypertension<ref>J Anat. Soc. India 49(2) 149-152 (2000). Available at: [http://www.indmedica.com/anatomy/aindex1.cfm?anid=41 http://www.indmedica.com/anatomy/aindex1.cfm?anid=41]. Accessed on: January 21, 2009.</ref>
***Membranous or velamentous (veil-like) insertion.
****Vessels separate/branch prior to reaching placental disc.
***Furcate insertion - blood vessels separate before reaching placenta disc/not surrounded by Wharton's jelly - vessels more exposed to trauma (risk for vasa previa).
**Knots (false vs. true).
***False knots are nothing to worry about -- look like a knot but aren't really one.
**Twisting/coiling - 1-3 coils/10 cm is normal.
**Number of vessels.
***Normal: 2 arteries, 1 vein.
*Membranes - shiny & translucent - normal (green, opaque/dull - chorioamnionitis).
**Attachment (insertion): marginal (normal), circummarginate (inside edge), [[circumvallate placenta|circumvallate]] (folding on self).
**Site of rupture - if obvious; low point of rupture suggests low-lying placenta.
*Placental disc.
**Fetal surface - normal is shinny.
***Dull in chorioamnionitis.
**Maternal surface
***Are the cotyledons intact?
***Adherent clot?
**Parenchyma - after sectioning:
***White vs. red nodules.

Notes:
*Parenchymal nodules - a brief DDx:
**White: [[placental infarct|infarct]] (chronic), thrombi, [[chorangioma]], [[perivillous fibrin deposition]].
**Red: infarct (acute), thrombi.

==Sections==
#Cord two sections.
#Membranes (rolled), two rolls or more.<ref name=pmid19061291>{{cite journal |author=Winters R, Waters BL |title=What is adequate sampling of extraplacental membranes?: a randomized, prospective analysis |journal=Arch. Pathol. Lab. Med. |volume=132 |issue=12 |pages=1920–3 |year=2008 |month=December |pmid=19061291 |doi= |url=}}</ref>
#Cord at insertion + disc.
#Placenta - full thickness (maternal and fetal surface).
#*Sections should not be taken at the margin of the disc.

==Placental membranes==
Appearance:<ref name=Ref_Lester461>{{Ref Lester|461}}</ref>
*Normal - shiny.
*[[Chorioamnionitis]] - opaque/dull.
*Meconium - green.
*[[Amnion nodosum]] - yellow patches.
**Some describe 'em as white.<ref>CS. 7 February 2011.</ref>

==Placental mass==
It is considered routine to obtain a mass for the placenta. This is usually done when the placenta is fresh and with the membranes and cord trimmed, as most tables of placental mass were created with these parameters.

Placental mass by gestational age:<ref>AFIP Placental pathol. ISBN: 1-881041-89-1. P.312</ref>
{| class="wikitable"
|Gest. Age/Percentile ||'''25%''' ||'''50%''' ||'''75%'''
|-
|'''32 weeks''' ||275 g ||318 g ||377 g
|-
|'''36 weeks''' ||369 g ||440 g ||508 g
|-
|'''40 weeks''' ||440 g ||501 g ||572 g
|-
|}

===Linear regression - placental mass-gestational age===
Based on the table in the AFIP book<ref>AFIP Placental pathol. ISBN: 1-881041-89-1. P.312</ref> I generated the following regression lines:
{| class="wikitable"
| ||'''50%''' ||'''10%''' ||'''90%'''
|-
|slope (g/week) ||21.58088235 ||19.70588235 ||25.40196078
|-
|y-intercept (g) ||-357.4558824 ||-397.2352941 ||-366.7254902
|-
|Pearson (r) ||0.988670724 ||0.988268672 ||0.982206408
|-
|}

placental mass = slope x gestational age + intercept

===What to remember...===
Extrapolated from the linear regression (see above):
*50% at term = 500 grams.
*50% at 26 weeks = 200 grams.
*The change in mass/week is approximately linear and equal to 300 grams / 14 weeks ~ 20 grams/week.
*The spread in mass between 10% and 90%, crudely estimated, is 200 grams (for GA=26-40).

Notes:
*Is it required?
**Sebire and Fox have advocated abandoning the practise of obtaining a placental mass, due to the large number of uncontrolled variables inherent in these measures. Instead, they have advocated using mushy descriptors such as "small", "average" and "large", which require experience in examining the organ.<ref>{{cite book |author= Fox, Harold; Sebire, Neil J. |title=[http://www.amazon.com/Pathology-Placenta-Major-Problems/dp/1416025928/ref=sr_1_fkmr0_1?ie=UTF8&qid=1297259619&sr=1-1-fkmr0 Pathology of the Placenta (Major Problems in Pathology)]|publisher=Saunders |location= |year=2007 |pages= 559-561 |edition=3rd |isbn=978-1416025924 |oclc= |doi= |accessdate=}}</ref>
***In the context of quality, a measure (even if somewhat flawed), is almost certainly more reproducible than arbitrary descriptors which require experience and a continuing case volume to calibrate.

===Placentomegaly===
*[[AKA]] ''large placenta''.
Associations:<ref>URL: [http://quizlet.com/5793113/ob-flash-cards/ http://quizlet.com/5793113/ob-flash-cards/]. Accessed on: 13 January 2012.</ref>
*Maternal [[diabetes]] - esp. poorly controlled.<ref name=pmid2771897>{{Cite journal | last1 = Clarson | first1 = C. | last2 = Tevaarwerk | first2 = GJ. | last3 = Harding | first3 = PG. | last4 = Chance | first4 = GW. | last5 = Haust | first5 = MD. | title = Placental weight in diabetic pregnancies. | journal = Placenta | volume = 10 | issue = 3 | pages = 275-81 | month = | year = | doi = | PMID = 2771897 }}</ref>
*Maternal [[anemia]]/low maternal iron stores.<ref>{{Cite journal | last1 = Hindmarsh | first1 = PC. | last2 = Geary | first2 = MP. | last3 = Rodeck | first3 = CH. | last4 = Jackson | first4 = MR. | last5 = Kingdom | first5 = JC. | title = Effect of early maternal iron stores on placental weight and structure. | journal = Lancet | volume = 356 | issue = 9231 | pages = 719-23 | month = Aug | year = 2000 | doi = | PMID = 11085691 }}</ref>
*Fetal malformations.
*Neoplasms of the placenta, e.g. [[chorangioma]].
*Twin-twin transfusion syndrome.
*Chronic intrauterine infections, e.g. [[syphilis]], [[toxoplasmosis]], [[cytomegalovirus]].

Lame causes of a heavy placenta:
*Dates wrong - error in determining the estimated date of confinement.
*Adherent blood clot.

Comment:
*Most of causes seem to have one thing in common:
**There is a decreased oxygen delivery to the fetus.

====Sign out====
<pre>
PLACENTA, UMBILICAL CORD AND FETAL MEMBRANES, CESAERIAN SECTION:
- LARGE PLACENTA (819 GRAMS -- TRIMMED, POST-FIXATION WEIGHT).
- FETAL MEMBRANES WITHIN NORMAL LIMITS.
- THREE VESSEL UMBILICAL CORD WITHIN NORMAL LIMITS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI.
</pre>

===Placental growth restriction===
*[[AKA]] ''placenta small for gestational age''.
*''Small placenta'' redirects here.
Associations:
*Maternal vascular disease, e.g. [[hypertrophic decidual vasculopathy|hypertension]].
*Fetal malformations.

====Sign out====
<pre>
PLACENTA, UMBILICAL CORD AND FETAL MEMBRANES, BIRTH:
- PLACENTA SMALL FOR GESTATIONAL AGE (160 GRAMS -- TRIMMED, POST-FIXATION WEIGHT).
- THREE VESSEL UMBILICAL CORD WITHIN NORMAL LIMITS.
- FETAL MEMBRANES WITHIN NORMAL LIMITS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI WITH:
-- OLD CENTRAL TRANSMURAL INFARCT (1.7 CM MAXIMAL DIMENSION).

COMMENT:
The 10th percentile placental mass (pre-fixation) for 34 weeks and 2 days is approximately 390 grams.
</pre>

=Overview of placental pathology=
==Approach==
The pathology of the placenta is diverse and is not easy to group.

It terms of remembering things. It is probably easiest to take a combined anatomical, etiologic and morphologic approach.

Anatomical basis:
*Cord.
*Membranes.
*Disc.

Etiologic:
*Congential.
*Infectious.
*Neoplastic.
*Endocrine.
*Trauma.
*Vascular.
*Degenerative.
*Autoimmune.
*Toxic.
*Idiopathic.

Compartmental:
*Vasculature.
*Membranes.
*Parenchyma:
**Maternal part (decidua).
**Fetal part (villi, cord).

==Common entities/diagnoses==
*Normal.
*[[Chorioamnionitis]].
*[[Placental abruption]].
*[[Meconium]].
*Hypertensive changes.

=Sign out=
What should be commented on...

*Placenta:
**Maturity of villi (2nd or 3rd trimester).
**Infarction?
***Subchorionic less important than maternal aspect.
***Peripheral aspect of placental disc less important than central region of disc.
**Blood vessels.
***Maternal.
***Fetal.
*Membranes.
**[[Membranitis]]?
**[[Chorioamnionitis]]?
*Cord:
**3 vessel?
**Vasculitis/inflammation?

Mnemonic: ''chorio, cord, vessels, villi (maturity, infarction)''.

==Normal placenta==
<pre>
PLACENTA, UMBILICAL CORD AND FETAL MEMBRANES, BIRTH:
- THREE VESSEL UMBILICAL CORD WITHIN NORMAL LIMITS.
- FETAL MEMBRANES WITHIN NORMAL LIMITS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI WITHIN NORMAL LIMITS.
</pre>

===C-section===
<pre>
PLACENTA, UMBILICAL CORD AND FETAL MEMBRANES, CAESAREAN SECTION:
- THREE VESSEL UMBILICAL CORD WITHIN NORMAL LIMITS.
- FETAL MEMBRANES WITHIN NORMAL LIMITS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI WITHIN NORMAL LIMITS.
</pre>

=Cord pathology=
*[[Two vessel cord]].
*Hypercoiling/Hypocoiling.
*Abnormal insertion.
*[[Cord knot]]s (true vs. false).
*Strictures.
*Hematoma.
*[[Hemangioma]].
*Benign cyst.

==Two vessel umbilical cord==
*[[AKA]] ''two vessel cord''.
*[[AKA]] ''single umbilical artery''.
{{Main|Two vessel umbilical cord}}

==Insertion==
===Marginal insertion===
Definition:
*The umbilical cord is attached to the placental disc at its margin.

Prevalence:
*Approximately 12% of placentas.<ref name=Ref_WMSP464>{{Ref WMSP|464}}</ref>

Relevance:
*None according to WMSP.<ref name=Ref_WMSP464>{{Ref WMSP|464}}</ref>
**In theory, the cord, dependent on its relation to the internal os, is at greater risk of injury (leading to vasa previa) and compression (fetal hypoxia). A retrospective study found cord position in relation to the internal os is predictive for vasa previa.<ref name=pmid20872421>{{cite journal |author=Hasegawa J, Farina A, Nakamura M, ''et al.'' |title=Analysis of the ultrasonographic findings predictive of vasa previa |journal=Prenat. Diagn. |volume=30 |issue=12-13 |pages=1121–5 |year=2010 |month=December |pmid=20872421 |doi=10.1002/pd.2618 |url=}}</ref>

===Velamentous insertion===
Definition:
*The umbilical cord inserts into the fetal membranes.<ref name=Ref_WMSP464>{{Ref WMSP|464}}</ref>
**The vessels are ''not'' protected by Wharton's jelly.
***Wharton's jelly = the connective tissue surrounding the vessels in the cord.

Details:<ref name=Ref_WMSP464>{{Ref WMSP|464}}</ref>
*3/4 of the time the vessel also branch; in the remaining 1/4 the vessels stay together.

Relevance:
*Increased risk of vasa previa.<ref name=pmid20872421>{{cite journal |author=Hasegawa J, Farina A, Nakamura M, ''et al.'' |title=Analysis of the ultrasonographic findings predictive of vasa previa |journal=Prenat. Diagn. |volume=30 |issue=12-13 |pages=1121–5 |year=2010 |month=December |pmid=20872421 |doi=10.1002/pd.2618 |url=}}</ref>

====Sign out====
<pre>
PLACENTA, UMBILICAL CORDS AND FETAL MEMBRANES, BIRTH:
- THREE VESSEL UMBILICAL CORD WITH A VELAMENTOUS INSERTION, OTHERWISE WITHIN NORMAL LIMITS.
- FETAL MEMBRANES WITHIN NORMAL LIMITS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI WITHIN NORMAL LIMITS.
</pre>

==Umbilical knot==
*[[AKA]] ''umbilical cord knot''.
*[[AKA]] ''cord knot''.
*[[AKA]] ''true knot''.
===General===
*Prevalence ~1.25%.<ref name=pmid12012287>{{cite journal |author=Airas U, Heinonen S |title=Clinical significance of true umbilical knots: a population-based analysis |journal=Am J Perinatol |volume=19 |issue=3 |pages=127–32 |year=2002 |month=April |pmid=12012287 |doi=10.1055/s-2002-25311 |url=}}</ref><ref name=Ref_WMSP>{{Ref WMSP|464}}</ref>
*Increase risk of [[stillbirth]]; odds ratio 3.93.<ref name=pmid12012287/>

===Gross===
Work-up:<ref name=Ref_WMSP>{{Ref WMSP|464}}</ref>
*Diameter measures and colour on both sides of the knot.
*Knot should be untied to assess for deformation of Wharton's jelly.
*Sections from both sides of the knot - to look for thrombi.

Note:
*False knots (large diameter - focally) are common - they cannot be untied.

===Microscopic===
Features:
*+/-[[thrombosis|Thrombi]].
**Fibrin deposition.
*+/-Lines of Zahn.

Images:
*[http://library.med.utah.edu/WebPath/ATHHTML/ATH031.html Lines of Zahn (utah.edu)].
*[http://pathhsw5m54.ucsf.edu/case9/image94.html Lines of Zahn (ucsf.edu)].

==Coiling==
*Hypo- and hypercoiling are both considered problematic.<ref name=Ref_WMSP464>{{Ref WMSP|464}}</ref>
**Normal: 1-3 coils/10 cm.<ref>CS. 7 February 2011.</ref>
*Associated with cord stricture, which is usu. at the fetal end of the cord.<ref name=Ref_WMSP465>{{Ref WMSP|465}}</ref>

Notes:
*There is little uniformity in how coiling is assessed in the medical literature - though 10% and 90% are considered the cut-points for normal.<ref name=pmid21080869>{{cite journal |author=Khong TY |title=Evidence-based pathology: umbilical cord coiling |journal=Pathology |volume=42 |issue=7 |pages=618–22 |year=2010 |month=December |pmid=21080869 |doi=10.3109/00313025.2010.520309 |url=}}</ref>
**What are the 10% and 90% cut-points? They are not given in WMSP. UT access to a journal article<ref name=pmid16076615>PMID 16076615.</ref> that might have it is screwed-up.

==Cord hematoma==
Features:<ref name=Ref_WMSP465>{{Ref WMSP|465}}</ref>
*Rare ~ 1/5500.
*Mortality ~50% is severe.

=Membranes=
*Squamous metaplasia.
*[[Chorioamnionitis]] - see ''infection'' section.

==Amnion nodosum==
{{Main|Amnion nodosum}}

==Placental meconium==
{{Main|Placental meconium}}

==Squamous metaplasia of the amnion==
===General===
*Benign common finding thought to be of no clinical significance.<ref name=Ref_WMSP463>{{Ref WMSP|463}}</ref>
**One case report suggesting an association with [[chorioamnionitis]].<ref>{{Cite journal | last1 = Chew | first1 = RH. | last2 = Silberberg | first2 = BK. | title = Possible association of acute inflammatory exudate in chorioamnionitis and amniotic squamous metaplasia. | journal = Am J Clin Pathol | volume = 93 | issue = 4 | pages = 582-5 | month = Apr | year = 1990 | doi = | PMID = 2321592 }}</ref>
*Needs to be separated from amnion nodosum - '''important'''.<ref>CS. 7 February 2011.</ref>

===Gross===
Features:<ref name=pmid18081444>{{Cite journal | last1 = Adeniran | first1 = AJ. | last2 = Stanek | first2 = J. | title = Amnion nodosum revisited: clinicopathologic and placental correlations. | journal = Arch Pathol Lab Med | volume = 131 | issue = 12 | pages = 1829-33 | month = Dec | year = 2007 | doi = 10.1043/1543-2165(2007)131[1829:ANRCAP]2.0.CO;2 | PMID = 18081444 }}</ref>
*White (or yellow) plaques - irregular outline.

DDx:
*[[Amnion nodosum]] - small (~1-5 mm), round, classically yellow.

Images:
*[http://www.archivesofpathology.org/action/showFullPopup?id=i1543-2165-131-12-1829-f01&doi=10.1043%2F1543-2165%282007%29131%5B1829%3AANRCAP%5D2.0.CO%3B2 Amnion nodosum & squamous metaplasia of the amnion (archivesofpathology.org)].<ref name=pmid18081444/>


===Microscopic===
Features:<ref name=pmid18081444/>
*Dense, paucicellular (pink) compact keratin - '''key feature'''.

Image:
*[http://flylib.com/books/2/953/1/html/2/43%20-%20Placenta_files/DA10C43FF29.png Squmous metaplasia of the amnion (flylib.com)].<ref>URL: [http://flylib.com/books/en/2.953.1.49/1/ http://flylib.com/books/en/2.953.1.49/1/]. Accessed on: 10 January 2011.</ref>

==Circumvallate placenta==
*[[AKA]] ''circumvallate insertion of the membranes''.
===General===
*May be associated with [[placental abruption]].<ref name=pmid18226129>{{Cite journal | last1 = Suzuki | first1 = S. | title = Clinical significance of pregnancies with circumvallate placenta. | journal = J Obstet Gynaecol Res | volume = 34 | issue = 1 | pages = 51-4 | month = Feb | year = 2008 | doi = 10.1111/j.1447-0756.2007.00682.x | PMID = 18226129 }}</ref>

Note:
*Membranes usually attach to the edge of the placenta.

===Gross===
*Fetal membranes attach to the fetal surface of the placenta away from the margin of the placental disc.

Classification:
*Partial - not circumferential.
*Complete.

DDx:
*[[Circummarginate placenta]].

Images:
*[http://library.med.utah.edu/nmw/mod2/Tutorial2/pics/circumvallate.jpg Circumvallate placenta - partial and complete (utah.edu)].
*[http://library.med.utah.edu/WebPath/jpeg2/PLAC027.jpg Circumvallate placenta (utah.edu)].

=Twin placentas=
{{Main|Twin placentas}}
These are often submitted... even if they are normal. In these specimens, usually, the chorion is the key.

It covers:
*Monozygotic vs. dizygotic twins.
*Twin-to-twin transfusion syndrome.

=Placental disc=
==Villous edema==
===General===
*Non-specific finding.
*Reported in associated with congenital adrenal hyperplasia for the stem villi.<ref name=pmid11045335>{{Cite journal | last1 = Furuhashi | first1 = M. | last2 = Oda | first2 = H. | last3 = Nakashima | first3 = T. | title = Hydrops of placental stem villi complicated with fetal congenital adrenal hyperplasia. | journal = Arch Gynecol Obstet | volume = 264 | issue = 2 | pages = 101-4 | month = Sep | year = 2000 | doi = | PMID = 11045335 }}</ref>

===Microscopic===
Features:
*"Swiss chesse-like" appearance / bubbly appearance.
*Usually patchy and focal.

Note:
*Cistern formation is reported in the stem villi in association with congenital adrenal hyperplasia.<ref name=pmid11045335/>

DDx:
*[[Chorioamnionitis]].
*Fetal edema.
*Idiopathic (no cause apparent).
*[[Placental villous immaturity]].

Image:
*[http://www.med.yale.edu/obgyn/kliman/placenta/articles/EOR_Placenta/Image19.gif villous edema (yale.edu)].<ref>URL: [http://www.med.yale.edu/obgyn/kliman/placenta/articles/EOR_Placenta/Trophtoplacenta.html http://www.med.yale.edu/obgyn/kliman/placenta/articles/EOR_Placenta/Trophtoplacenta.html]. Accessed on: 28 May 2011.</ref>

==Placental villous immaturity==
{{Main|Placental villous immaturity}}

==Villous hypoplasia==
*[[AKA]] ''terminal villus deficiency''.<ref name=Ref_Placenta346>{{Ref Placenta|346}}</ref>
{{Main|Villous hypoplasia}}

=Diseases of the placental attachment=
==Placenta creta==
Includes ''placenta accreta'', ''placenta increta'', and ''placenta percreta''.
{{Main|Placenta creta}}

==Placental abruption==
{{Main|Placental abruption}}

=Inflammatory pathologies=
===Overview of infections===
General:<ref name=Ref_PBoD1106>{{Ref PBoD|1106}}</ref>
*Infection usually ascending, i.e. from vagina up through cervix.
**Associated with intercourse.
*Hematogenous rare - manifest as villitis.
**Think ''[[TORCH infections]]'' ([[toxoplasmosis]], others ([[syphilis]], [[TB]], listeriosis), rubella, [[cytomegalovirus]], [[herpes simplex virus]]).
*Funisitis usually follows chorioamnionitis.
**Inflammatory cells in umbilical cord are fetal (trivia).

====Types====
By site:<ref name=Ref_PBoD1106>{{Ref PBoD|1106}}</ref>
*Fetal membranes: chorioamnionitis, membranitis.<ref name=Ref_Sternberg4_2311>{{Ref Sternberg4|2311}}</ref>
*Umbilical cord: funisitis.
*Placenta: placentitis, villitis.

==Membranitis==
:''Chorionitis'' redirects here.
===General===
*Early [[chorioamnionitis]].<ref>{{Cite journal | last1 = Vedovato | first1 = S. | last2 = Zanardo | first2 = V. | title = [Chorioamnionitis and inflammatory disease in the premature newborn infant]. | journal = Minerva Pediatr | volume = 62 | issue = 3 Suppl 1 | pages = 155-6 | month = Jun | year = 2010 | doi = | PMID = 21090086 }}</ref>
*Controversial.{{fact}}

===Microscopic===
Features:
*[[PMN]]s in the decidua.
*+/-PMNs in subamniotic tissue.
*+/-Necrosis in decidua or chorion/subamniotic tissue.

Note:
*Plasma cells in the decidua = [[chronic deciduitis]].

DDx:
*[[Chorioamnionitis]].

====Grading membranitis====
''Sternberg'':<ref name=Ref_Sternberg4_2311>{{Ref Sternberg4|2311}}</ref>
# PMNs - decidua only.
# PMNs - in subamniotic tissue.
# 1 or 2 + [[necrosis]] in decidua or chorion/subamniotic tissue.

===Sign out===
<pre>
PLACENTA, UMBILICAL CORD AND FETAL MEMBRANES, CESAERIAN SECTION:
- FETAL MEMBRANES WITH CHORIONITIS.
- THREE VESSEL UMBILICAL CORD WITH VASCULITIS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI.
</pre>

====Waffle====
<pre>
PLACENTA, UMBILICAL CORD AND FETAL MEMBRANES, BIRTH:
- FETAL MEMBRANES WITH MECONIUM-LADEN MACROPHAGES AND ABUNDANT DECIDUAL NEUTROPHILS
SUSPICIOUS FOR EARLY CHORIONITIS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI.
- THREE VESSEL UMBILICAL CORD WITHIN NORMAL LIMITS.
</pre>

==Chorioamnionitis==
{{Main|Chorioamnionitis}}

==Umbilical cord vasculitis==
{{Main|Umbilical cord vasculitis}}

==Funisitis==
{{Main|Funisitis}}
*Inflammation of Wharton's jelly - the connective tissue of the umbilical cord.

==Acute villitis==
{{main|Acute villitis}}

==Villitis of unknown etiology==
{{Main|Villitis of unknown etiology}}

==Chronic intervillitis==
*[[AKA]] ''chronic intervillositis''.<ref name=pmid8215826>{{Cite journal | last1 = Jacques | first1 = SM. | last2 = Qureshi | first2 = F. | title = Chronic intervillositis of the placenta. | journal = Arch Pathol Lab Med | volume = 117 | issue = 10 | pages = 1032-5 | month = Oct | year = 1993 | doi = | PMID = 8215826 }}</ref>

===General===
*Rare.
*Massive chronic intervillitis - associated [[IUGR]], spontaneous abortion, perinatal fetal death.<ref name=pmid17088773>{{Cite journal | last1 = Rota | first1 = C. | last2 = Carles | first2 = D. | last3 = Schaeffer | first3 = V. | last4 = Guyon | first4 = F. | last5 = Saura | first5 = R. | last6 = Horovitz | first6 = J. | title = [Perinatal prognosis of pregnancies complicated by placental chronic intervillitis]. | journal = J Gynecol Obstet Biol Reprod (Paris) | volume = 35 | issue = 7 | pages = 711-9 | month = Nov | year = 2006 | doi = | PMID = 17088773 }}</ref>
*Recurs.
===Microscopic===
Features:<ref name=pmid8215826/><ref name=pmid17088773/>
*Intervillous inflammatory cells:
**Lymphocytes.
**Histiocytes.
*Fibrinoid deposition.

====Images====
<gallery>
Image:Intervillitis_-_intermed_mag.jpg | Intervillitis - intermed. mag. (WC)
Image:Intervillitis_-_very_high_mag.jpg | Intervillitis - very high mag. (WC)
</gallery>
==Chronic deciduitis==
*[[AKA]] plasma cell deciduitis.
{{Main|Chronic deciduitis}}

=Placental infarction=
==True infarcts==
{{Main|Placental infarct}}

==Perivillous fibrin deposition==
*Abbreviation ''PFD''.
===General===
*Thought to be an immunologic problem - resulting in platelet activation and fibrin deposition.<ref name=pmid12066949>{{Cite journal | last1 = Sebire | first1 = NJ. | last2 = Backos | first2 = M. | last3 = Goldin | first3 = RD. | last4 = Regan | first4 = L. | title = Placental massive perivillous fibrin deposition associated with antiphospholipid antibody syndrome. | journal = BJOG | volume = 109 | issue = 5 | pages = 570-3 | month = May | year = 2002 | doi = | PMID = 12066949 }}</ref>
*May be associated with [[diabetes mellitus]].<ref name=Ref_Placenta327>{{Ref Placenta|327}}</ref>

===Gross===
*Pale (white).
*Firm.
*White fibrous sepatae.

===Microscopic===
Features:
*Acellular eosinophilic material around formed villi.
*Obliteration of intervillous space.
**Intervillous distance increased vis-a-vis normal - '''key feature'''.

Notes:
*Nuclei of villi are usually preserved.
*Villi may have secondary infarction, i.e. there may be [[Basics#Nuclear destruction words|nuclear destruction]] (karyolysis, karyorrhexis, pyknosis).

DDx:
*[[Placental infarction]] - loss of nuclei in the villi (below the edge of the lesion).
*[[Massive perivillous fibrin deposition]] (maternal floor infarct).

Images:
*[http://path.upmc.edu/cases/case75.html APLA syndrome (upmc.edu)].

===Sign out===
====Thrombi====
<pre>
PLACENTA, UMBILICAL CORD AND FETAL MEMBRANES, BIRTH:
- THREE VESSEL UMBILICAL CORD WITHIN NORMAL LIMITS.
- FETAL MEMBRANES WITHIN NORMAL LIMITS.
- PLACENTAL DISC WITH THIRD TRIMESTER VILLI AND THREE LARGE INTERVILLOUS
THROMBI (BLOCKS A7-A9).
</pre>

==Maternal floor infarction==
*Abbreviated ''MFI''.
*Formally ''placental maternal floor infarction''.
*[[AKA]] ''massive perivillous fibrin deposition''.<ref name=Ref_Placenta367>{{Ref Placenta|367}}</ref>
{{Main|Maternal floor infarction}}

=Fetal disease=
==Fetal thrombotic vasculopathy==
*Abbreviated ''FTV''.
*A large number of terms are used for this including:<ref name=pmid19237859>{{Cite journal | last1 = Marchetti | first1 = D. | last2 = Belviso | first2 = M. | last3 = Fulcheri | first3 = E. | title = A case of stillbirth: the importance of placental investigation in medico-legal practice. | journal = Am J Forensic Med Pathol | volume = 30 | issue = 1 | pages = 64-8 | month = Mar | year = 2009 | doi = 10.1097/PAF.0b013e318187387e | PMID = 19237859 }}</ref>
**''Fibrinous vasculosis''.
**''Fibromuscular sclerosis''.
**''Fetal artery stem thrombosis''.
*The multitude of terms reflects the confusion about this finding and that it has numerous etiologies.<ref name=pmid19237859/>
{{Main|Fetal thrombotic vasculopathy}}

==Hemorrhagic endovasculitis==
*Abbreviated ''HEV''.
===General===
*Associated with stillbirth.<ref name=pmid6151926>{{cite journal |author=Stevens NG, Sander CH |title=Placental hemorrhagic endovasculitis: risk factors and impact on pregnancy outcome |journal=Int J Gynaecol Obstet |volume=22 |issue=5 |pages=393–7 |year=1984 |month=October |pmid=6151926 |doi= |url=}}</ref>

===Microscopic===
Features:<ref name=pmid11825110>{{cite journal |author=Sander CM, Gilliland D, Akers C, McGrath A, Bismar TA, Swart-Hills LA |title=Livebirths with placental hemorrhagic endovasculitis: interlesional relationships and perinatal outcomes |journal=Arch. Pathol. Lab. Med. |volume=126 |issue=2 |pages=157–64 |year=2002 |month=February |pmid=11825110 |doi= |url=}}</ref>
*Walls of the (fetal) placental blood vessels (in the villi) are disrupted.
*+/-Intraluminal necrotic debris.
*[[RBC]] fragmentation.

=Maternal disease=
==Hypertensive changes==
===General===
Associated pathologic changes:<ref name=pmid6754249>{{cite journal |author=Soma H, Yoshida K, Mukaida T, Tabuchi Y |title=Morphologic changes in the hypertensive placenta |journal=Contrib Gynecol Obstet |volume=9 |issue= |pages=58–75 |year=1982 |pmid=6754249 |doi= |url=}}</ref>
*Placental infarcts.
*Increased syncytial knots.
*Hypovascularity of the villi.
*Cytotrophoblastic proliferation.
*Thickening of the trophoblastic basement membrane.

===Microscopic===
Features:<ref name=pmid6754249>{{cite journal |author=Soma H, Yoshida K, Mukaida T, Tabuchi Y |title=Morphologic changes in the hypertensive placenta |journal=Contrib Gynecol Obstet |volume=9 |issue= |pages=58–75 |year=1982 |pmid=6754249 |doi= |url=}}</ref>
*Enlarged endothelial cells - fetal capillaries.
*Atherosis of the spiral arteries - placental bed (maternal).

Notes:
*One should look for the changes in the membrane roll, not the maternal surface.<ref>Sherman, C. 7 February 2011.</ref>

Images:
*[http://www.pathxchange.org/case/19711 Pregnancy-induced hypertension (pathxchange.org)].

==Hypertrophic decidual vasculopathy==
:[[AKA]] ''decidual vasculopathy''.
{{Main|Hypertrophic decidual vasculopathy}}

==HELLP syndrome==
{{Main|HELLP syndrome}}

==Malaria==
{{Main|Malaria}}
===General===
*Uncommon in Canada.
*May lead to fetal demise.

===Microscopic===
Feature:
*[[RBC]]s with basophilic dots ~1-2 micrometres.

====Image====
<gallery>
Image:Maternal_malaria_placenta_-_very_high_mag.jpg | Maternal malaria - very high mag. (WC)
</gallery>
=Tumours=
{{main|Gestational trophoblastic disease}}

==Chorangioma==
{{Main|Chorangioma}}

==Chorangiomatosis==
===General===
Associated with:
*Preeclampsia.
*[[IUGR]].

===Gross===
*Multiple tan nodules.

===Microscopic===
Features:
*Multiple chorangiomas - the difference between chorangioma and chorangiomatosis is not well defined.<ref>URL: [http://path.upmc.edu/cases/case655/dx.html http://path.upmc.edu/cases/case655/dx.html]. Accessed on: 28 January 2012.</ref>

Images:
*[http://path.upmc.edu/cases/case655.html Chorangiomatosis - several images (upmc.edu)].

==Chorangiosis==
{{Main|Chorangiosis}}

=Other=
==Fetus papyraceus==
*May be spelled ''foetus papyraceus''.
*[[AKA]] ''fetus compressus''.
{{Main|Fetus papyraceus}}

==Placental mesenchymal dysplasia==
*Abbreviated ''PMD''.
{{Main|Placental mesenchymal dysplasia}}

=Placental cysts and pseudocysts=
Types:<ref name=Ref_Placenta219-220>{{Ref Placenta|219-220}}</ref>
*Amnionic epithelial inclusion cyst (amniotic cyst).
*[[Epidermal inclusion cyst]] - lined by keratinized squamous epithelium.
*Chorionic cyst ([[AKA]] chorionic pseudocyts).
*Cell island cyst.

Other considerations:<ref name=pmid12054300>{{Cite journal | last1 = Brown | first1 = DL. | last2 = DiSalvo | first2 = DN. | last3 = Frates | first3 = MC. | last4 = Davidson | first4 = KM. | last5 = Genest | first5 = DR. | title = Placental surface cysts detected on sonography: histologic and clinical correlation. | journal = J Ultrasound Med | volume = 21 | issue = 6 | pages = 641-6; quiz 647-8 | month = Jun | year = 2002 | doi = | PMID = 12054300 }}</ref>
*Hematoma.
*Fibrin-lined pseudocyst.

General:<ref name=pmid12054300/>
*Usually good outcome.
*Large cysts (>4.5 cm) or multiple cysts (>3) are associated with [[IUGR]].

Images:
*[http://www.jultrasoundmed.org/content/21/6/641/F5.expansion.html Subchorionic cysts (jultrasoundmed.org)].<ref name=pmid12054300/>

=See also=
*[[Chorionic villi]].
*[[Endometrium]].
*[[Pregnancy]].
*[[Gestational trophoblastic disease]].
*[[TORCH infections]].

=References=
{{reflist|2}}

=Recommended reading=
*{{cite journal |author=Langston C, Kaplan C, Macpherson T, ''et al.'' |title=Practice guideline for examination of the placenta: developed by the Placental Pathology Practice Guideline Development Task Force of the College of American Pathologists |journal=Arch. Pathol. Lab. Med. |volume=121 |issue=5 |pages=449–76 |year=1997 |month=May |pmid=9167599 |doi= |url=}}
*{{cite book |author= Baergen, Rebecca N. |title=[http://www.amazon.com/Manual-Benirschke-Kaufmanns-Pathology-Placenta/dp/0387220895/ref=sr_1_1?ie=UTF8&qid=1297169019&sr=8-1 Manual of Benirschke and Kaufmann's Pathology of the Human Placenta] |publisher=Springer |location= |year=2005 |pages= {{{1|}}} |edition=1st |isbn=978-0387220895 |oclc= |doi= |accessdate=}}

=External links=
*[http://emedicine.medscape.com/article/262470-overview Cord complications (emedicine.medscape.com)].
*[http://www.palpath.com/MedicalTestPages/placenta2.htm Placenta notes (palpath.com)].

[[Category:Placenta]]

Fibroblastic/myofibroblastic tumours

2018-05-10T00:59:50Z

Brigitte: /* Microscopic */

This article covers '''fibroblastic/myofibroblastic tumours'''. These tumours fit into the larger category of [[soft tissue lesions]].

=List of tumours=

===Benign===
WHO classification:<ref name=Ref_WMSP601>{{Ref WMSP|601}}</ref>
*[[Nodular fasciitis]].
*[[Proliferative fasciitis]].
*[[Proliferative myositis]].
*[[Myositis ossificans]].
*Ischemic fasciitis.
*[[Elastofibroma]].
*[[Myofibroma]].
*Fibromatosis colli. 
*Inclusion body fibromatosis.
*Fibroma of tendon sheath.
*Calcifying aponeurotic fibroma.
*[[Angiomyofibroblastoma]].
*[[Cellular angiofibroma]].
*Nuchal-type fibroma.
*Gardner fibroma.
*[[Calcifying fibrous tumour]].
*Giant cell angiofibroma.

*Fibrous hamartoma of infancy.
*Juvenile hyaline fibromatosis.
*[[Desmoplastic fibroblastoma]].
*Mammary-type myofibroblastoma.

===Locally aggressive===
WHO classification:<ref name=Ref_WMSP601>{{Ref WMSP|601}}</ref>
*Superfical fibromatosis.
*[[Desmoid-type fibromatosis]].
*[[Lipofibromatosis]].

===Occasionally metastasizing===
WHO classification:<ref name=Ref_WMSP602>{{Ref WMSP|602}}</ref>
*[[Solitary fibrous tumour]].
*[[Inflammatory myofibroblastic tumour]].
*[[Low-grade myofibroblastic sarcoma]].
*Myxoinflammatory fibroblastic sarcoma.
*[[Infantile fibrosarcoma]].

===Malignant===
WHO classification:<ref name=Ref_WMSP602>{{Ref WMSP|602}}</ref>
*[[Adult fibrosarcoma]].
*[[Myxofibrosarcoma]].
*[[Low-grade fibromyxoid sarcoma]] (hyalinizing spindle cell tumour).
*Sclerosing epithelioid fibrosarcoma.

=Non-malignant=
==Proliferative fasciitis==
===General===
*Benign.
*May mimic a sarcoma.<ref name=pmid1058047>{{Cite journal | last1 = Chung | first1 = EB. | last2 = Enzinger | first2 = FM. | title = Proliferative fasciitis. | journal = Cancer | volume = 36 | issue = 4 | pages = 1450-8 | month = Oct | year = 1975 | doi = | PMID = 1058047 }}</ref>

Clinical:
*Solid subcutaneous nodule.
*Rapid growth.
*May be painful.

===Gross===
*Classically upper and lower extremities.<ref name=pmid1058047/>
*Poorly demarcated.

===Microscopic===
Features:<ref name=pmid1566969>{{Cite journal | last1 = Meis | first1 = JM. | last2 = Enzinger | first2 = FM. | title = Proliferative fasciitis and myositis of childhood. | journal = Am J Surg Pathol | volume = 16 | issue = 4 | pages = 364-72 | month = Apr | year = 1992 | doi = | PMID = 1566969 }}</ref>
*Large polygonal (ganglion-like) and/or spindled cells with:
**[[Vesicular nuclei|Vesicular (clear) nuclei]].
**Prominent nucleoli.
*+/-Binucleation.
*Loose myxoid stroma.
*Frequent typical mitoses.
**'''No''' atypical mitoses.

DDx:
*[[Inflammatory myofibroblastic tumour]].<ref name=pmid17938159>{{Cite journal | last1 = Gleason | first1 = BC. | last2 = Hornick | first2 = JL. | title = Inflammatory myofibroblastic tumours: where are we now? | journal = J Clin Pathol | volume = 61 | issue = 4 | pages = 428-37 | month = Apr | year = 2008 | doi = 10.1136/jcp.2007.049387 | PMID = 17938159 }}</ref>

Images:
*[http://www.medicine.virginia.edu/clinical/departments/pathology/Case%20Studies/known-pathology-cases/95_4-page Proliferative fasciitis - several images (virginia.edu)].
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090721114221299 Proliferative fasciitis (surgicalpathologyatlas.com)].

==Proliferative myositis==
===General===
*Benign.
*Possible arise from pericytes.<ref name=pmid2058761/>

===Microscopic===
Features:<ref name=pmid2058761>{{Cite journal | last1 = el-Jabbour | first1 = JN. | last2 = Bennett | first2 = MH. | last3 = Burke | first3 = MM. | last4 = Lessells | first4 = A. | last5 = O'Halloran | first5 = A. | title = Proliferative myositis. An immunohistochemical and ultrastructural study. | journal = Am J Surg Pathol | volume = 15 | issue = 7 | pages = 654-9 | month = Jul | year = 1991 | doi = | PMID = 2058761 }}</ref><ref name=pmid1586481>{{Cite journal | last1 = Lundgren | first1 = L. | last2 = Kindblom | first2 = LG. | last3 = Willems | first3 = J. | last4 = Falkmer | first4 = U. | last5 = Angervall | first5 = L. | title = Proliferative myositis and fasciitis. A light and electron microscopic, cytologic, DNA-cytometric and immunohistochemical study. | journal = APMIS | volume = 100 | issue = 5 | pages = 437-48 | month = May | year = 1992 | doi = | PMID = 1586481 }}</ref>
*Large ganglion-like cells.
**Cells have single prominent nucleolus.
*Spindle cells.
*+/-Binucleation.
*Mitotic activity.
**No atypical mitoses.

Image:
*[http://commons.wikimedia.org/wiki/File:Proliferative_myositis_%28HE%29.jpg Proliferative myositis (WC)].

===IHC===
Features:<ref name=pmid2058761/>
*Vimentin +ve.
*SMA +ve.
*Desmin +ve/-ve.

Others:<ref name=pmid2058761/>
*Factor XIIIa -ve.
*S100 -ve.
*CAM5.2 -ve.
*NSE -ve.

==Elastofibroma==
===General===
*Benign.
*Classically, subscapular in elderly women.<ref>URL: [http://emedicine.medscape.com/article/1057113-overview http://emedicine.medscape.com/article/1057113-overview]. Accessed on: 26 October 2011.</ref><ref>{{Cite journal | last1 = Ben Hassouna | first1 = J. | last2 = Hamdi | first2 = N. | last3 = Ben Bachouche | first3 = W. | last4 = Bouzid | first4 = T. | last5 = Dhiab | first5 = T. | last6 = Rahal | first6 = K. | title = Elastofibroma dorsi. | journal = Orthop Traumatol Surg Res | volume = 96 | issue = 6 | pages = 717-20 | month = Oct | year = 2010 | doi = 10.1016/j.otsr.2010.03.019 | PMID = 20708994 }}</ref>

===Gross===
Features:
*Yellow-white, moderate demarcation to surrounding tissue.<ref name=Ref_AoGP592>{{Ref AoGP|592}}</ref>

DDx - shoulder lesions:
*[[Desmoplastic fibroblastoma]].
*[[Pleomorphic lipoma]].

===Microscopic===
Features:
*Thick bundles of collagen.
*Elastin fibres.

Image:

==Nodular fasciitis==
{{Main|Nodular fasciitis}}

==Desmoid-type fibromatosis==
*[[AKA]] ''desmoid tumour''.
*[[AKA]] ''desmoid fibromatosis''.
{{Main|Desmoid-type fibromatosis}}

==Lipofibromatosis==
*[[AKA]] infantile subcutaneous fibromatosis.

===General===
*Childhood.

===Microscopic===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Fibroblastic cells surrounding adipocytes.

Image:
*[http://cases.edinburgh-dermatopathology.org.uk/#2.0 Lipofibromatosis (edinburgh-dermatopathology.org.uk)].

===IHC===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*CD34 +ve.
*BCL2 +ve.
*S100 +ve.
*CD99 +ve.
*Actin +ve.
*EMA +ve.

==Desmoplastic fibroblastoma==
*AKA ''collagenous fibroma''.<ref name=pmid18271804>{{Cite journal | last1 = Watanabe | first1 = H. | last2 = Ishida | first2 = Y. | last3 = Nagashima | first3 = K. | last4 = Makino | first4 = T. | last5 = Norisugi | first5 = O. | last6 = Shimizu | first6 = T. | title = Desmoplastic fibroblastoma (collagenous fibroma). | journal = J Dermatol | volume = 35 | issue = 2 | pages = 93-7 | month = Feb | year = 2008 | doi = 10.1111/j.1346-8138.2008.00421.x | PMID = 18271804 }}</ref>
*'''Not''' to be confused with ''[[desmoplastic fibroma]]''.
===General===
*Benign lesion.

Epidemiology:
*May be on the lip.
*Male:female ~= 5:1.<ref name=pmid15547225/>
*Age - typically 40s & 50s.<ref name=pmid15547225/>

===Gross===
*Classically found in the shoulder region.

DDx - shoulder region:
*[[Desmoplastic fibroblastoma]].
*[[Elastofibroma]].

===Microscopic===
Features:<ref name=pmid9670823/><ref name=Ref_Sternberg4_161>{{Ref Sternberg4|161}}</ref>
*Spindle cells ''or'' stellate cells without nuclear atypia.
*Acellular stroma with abundant collagen - '''key feature'''.
*+/-Myxoid areas.
*+/-Rare mitoses.

DDx:<ref name=pmid9670823/>
*[[Fibromatosis]].
*[[Low-grade fibromyxoid sarcoma]].

Images:
*[http://www.webpathology.com/image.asp?case=458&n=1 Desmoplastic fibroblastoma (webpathology.com)].
*[http://www.ajronline.org/content/183/6/1766/F3.expansion Desmoplastic fibroblastoma (ajronline.org)].<ref name=pmid15547225>{{Cite journal | last1 = Walker | first1 = KR. | last2 = Bui-Mansfield | first2 = LT. | last3 = Gering | first3 = SA. | last4 = Ranlett | first4 = RD. | title = Collagenous fibroma (desmoplastic fibroblastoma) of the shoulder. | journal = AJR Am J Roentgenol | volume = 183 | issue = 6 | pages = 1766 | month = Dec | year = 2004 | doi = | PMID = 15547225 }}</ref>

===IHC===
Features:<ref name=pmid9670823>{{Cite journal | last1 = Miettinen | first1 = M. | last2 = Fetsch | first2 = JF. | title = Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts. | journal = Hum Pathol | volume = 29 | issue = 7 | pages = 676-82 | month = Jul | year = 1998 | doi = | PMID = 9670823 }}</ref>
*Beta-catenin -ve.<ref name=pmid18544056>{{Cite journal | last1 = Takahara | first1 = M. | last2 = Ichikawa | first2 = R. | last3 = Oda | first3 = Y. | last4 = Uchi | first4 = H. | last5 = Takeuchi | first5 = S. | last6 = Moroi | first6 = Y. | last7 = Kiryu | first7 = H. | last8 = Furue | first8 = M. | title = Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis. | journal = J Cutan Pathol | volume = 35 Suppl 1 | issue = | pages = 70-3 | month = Oct | year = 2008 | doi = 10.1111/j.1600-0560.2007.00964.x | PMID = 18544056 }}
</ref>
**+ve in [[desmoid-type fibromatosis]].
*Desmin -ve.
*S-100 -ve.
*CD34 -ve.
*MSA +ve (focal).
*alpha-SMA +ve (focal).

===Molecular===
*llq12 breakpoint described as being characteristic -- possibly the ''FOSL1 gene''.<ref name=pmid22411068>{{Cite journal | last1 = Macchia | first1 = G. | last2 = Trombetta | first2 = D. | last3 = Möller | first3 = E. | last4 = Mertens | first4 = F. | last5 = Storlazzi | first5 = CT. | last6 = Debiec-Rychter | first6 = M. | last7 = Sciot | first7 = R. | last8 = Nord | first8 = KH. | title = FOSL1 as a candidate target gene for 11q12 rearrangements in desmoplastic fibroblastoma. | journal = Lab Invest | volume = 92 | issue = 5 | pages = 735-43 | month = May | year = 2012 | doi = 10.1038/labinvest.2012.46 | PMID = 22411068 }}</ref>

==Calcifying fibrous tumour==
===General===
*Rare.
*Benign.

===Microscopic===
Features:<ref name=pmid16858502>{{cite journal |author=Attila T, Chen D, Gardiner GW, Ptak TW, Marcon NE |title=Gastric calcifying fibrous tumor |journal=Can. J. Gastroenterol. |volume=20 |issue=7 |pages=487–9 |year=2006 |month=July |pmid=16858502 |pmc=2659917 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659917/}}</ref>
*Submucosal circumscribed fibrocollagenous nodule.
*Psammomatous calcifications.
*Focal plasma cells at the periphery.

==Myofibroma==
{{Main|Myofibroma}}

==Cellular angiofibroma==
===General===
*Rare.
*Benign.
*Probably related to [[spindle cell lipoma]] and [[mammary-type myofibroblastoma]].<ref name=pmid20852591>{{Cite journal | last1 = Flucke | first1 = U. | last2 = van Krieken | first2 = JH. | last3 = Mentzel | first3 = T. | title = Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. | journal = Mod Pathol | volume = 24 | issue = 1 | pages = 82-9 | month = Jan | year = 2011 | doi = 10.1038/modpathol.2010.170 | PMID = 20852591 }}</ref>
*Predominantly female.

===Gross===
Features:<ref name=pmid20852591/>
*Superficial.
*Well-circumscribed.

Classic location:
*Vulva.<ref name=pmid20852591/>

===Microscopic===
Features:<ref name=pmid20852591/>
*Spindle cell lesion.
*Many small-to-medium blood vessls.

===IHC===
Features:<ref name=pmid20852591/>
*CD34 ~50% of cases.
*SMA ~41% of cases.
*CD99 -ve.
*EMA -ve.

=Occasionally metastasizing=
==Inflammatory myofibroblastic tumour==
*[[AKA]] inflammatory pseudotumour, AKA inflammatory fibrosarcoma,<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref> AKA plasma cell granuloma.<ref>URL: [http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung]. Accessed on: 27 November 2011.</ref><ref name=pmid21772725>{{Cite journal | last1 = Manohar | first1 = B. | last2 = Bhuvaneshwari | first2 = S. | title = Plasma cell granuloma of gingiva. | journal = J Indian Soc Periodontol | volume = 15 | issue = 1 | pages = 64-6 | month = Jan | year = 2011 | doi = 10.4103/0972-124X.82275 | PMID = 21772725 }}</ref>
{{Main|Inflammatory myofibroblastic tumour}}

==Low-grade myofibroblastic sarcoma==
===General===
*Rare ~ 100 cases in the literature.
*Usu. oral cavity or extremities.<ref name=pmid21868549/>

===Microscopic===
Features:
*Spindle cells in the storiform pattern<ref name=pmid21868549/> ''or'' in fasicles.
*Rare mitoses.

Images:
*[http://path.upmc.edu/cases/case673.html Low-grade myofibroblastic sarcoma - several images (upmc.edu)].

DDx:
*Atypical [[leiomyoma]].
*[[GIST]].
*[[Leiomyosarcoma]].

===IHC===
*SMA +ve.
*CD34 -ve.
*CD117 -ve.<ref name=pmid21868549/>
*H-caldesmon -ve.<ref name=pmid21868549>{{Cite journal | last1 = Miyazawa | first1 = M. | last2 = Naritaka | first2 = Y. | last3 = Miyaki | first3 = A. | last4 = Asaka | first4 = S. | last5 = Isohata | first5 = N. | last6 = Yamaguchi | first6 = K. | last7 = Murayama | first7 = M. | last8 = Shimakawa | first8 = T. | last9 = Katsube | first9 = T. | title = A low-grade myofibroblastic sarcoma in the abdominal cavity. | journal = Anticancer Res | volume = 31 | issue = 9 | pages = 2989-94 | month = Sep | year = 2011 | doi = | PMID = }}</ref>

==Congenital-infantile fibrosarcoma==
:Should not be confused with ''[[adult fibrosarcoma]]''.
===General===
*Locally aggressive.

===Microscopic===
Features:<ref name=pmid7839472>{{Cite journal | last1 = Corsi | first1 = A. | last2 = Boldrini | first2 = R. | last3 = Bosman | first3 = C. | title = Congenital-infantile fibrosarcoma: study of two cases and review of the literature. | journal = Tumori | volume = 80 | issue = 5 | pages = 392-400 | month = Oct | year = 1994 | doi = | PMID = 7839472 }}</ref>
*Spindle cell lesion.

===Molecular===
Characteristic [[translocation]]:<ref>{{Cite journal | last1 = Sheng | first1 = WQ. | last2 = Hisaoka | first2 = M. | last3 = Okamoto | first3 = S. | last4 = Tanaka | first4 = A. | last5 = Meis-Kindblom | first5 = JM. | last6 = Kindblom | first6 = LG. | last7 = Ishida | first7 = T. | last8 = Nojima | first8 = T. | last9 = Hashimoto | first9 = H. | title = Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. | journal = Am J Clin Pathol | volume = 115 | issue = 3 | pages = 348-55 | month = Mar | year = 2001 | doi = 10.1309/3H24-E7T7-V37G-AKKQ | PMID = 11242790 }}</ref>
*t(12;15)(p13;q25).
**Gene fusion ETV6-NTRK3.
***Same translocation in [[mesoblastic nephroma]].

==Solitary fibrous tumour==
{{Main|Solitary fibrous tumour}}

==Hemangiopericytoma==
===General===
*Grouped with ''[[solitary fibrous tumour]]'' in the WHO classification; share same genetic NAB2-STAT6 fusion.<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref>{{Cite journal | last1 = Schweizer | first1 = L. | last2 = Koelsche | first2 = C. | last3 = Sahm | first3 = F. | last4 = Piro | first4 = RM. | last5 = Capper | first5 = D. | last6 = Reuss | first6 = DE. | last7 = Pusch | first7 = S. | last8 = Habel | first8 = A. | last9 = Meyer | first9 = J. | title = Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. | journal = Acta Neuropathol | volume = 125 | issue = 5 | pages = 651-8 | month = May | year = 2013 | doi = 10.1007/s00401-013-1117-6 | PMID = 23575898 }}</ref>

*Thought to arise from the ''pericyte'', a connective tissue cell of small vessels that is thought to be involved in flow regulation while others consider a fibroblastic nature.<ref>{{Cite journal | last1 = Gengler | first1 = C. | last2 = Guillou | first2 = L. | title = Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. | journal = Histopathology | volume = 48 | issue = 1 | pages = 63-74 | month = Jan | year = 2006 | doi = 10.1111/j.1365-2559.2005.02290.x | PMID = 16359538 }}</ref>
*Hematologic spread most common - to lungs.<ref name=emed1255879ov>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*[[Oncogenic osteomalacia]] - assoc. with hemangiopericytoma.<ref name=emed1255879ov>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*WHO grade II hemangiopericytoma (ICD-O: 9150/1), WHO grade III anaplastic hemangiopericytoma (ICD-O: 9150/3)

====Presentation====
*Usually painless mass, slow enlargement.
*May profusely bleed during resection.
*May invade bone.

====Histology====
*high cellular density.
*indistinct cell borders.
*random tumor cell orientation.
*little fibrosis.
*plenty reticulin.
*vascular with slit-like channels ("staghorn-like vessels").

====IHC====
* Vimentin +ve.
* CD34 +ve (often patchy, used to differentiate from SFT).
* [[STAT6]] nuclear +ve.
* EMA +/-ve.

===Radiology===
*Intramedullary lytic mass.
*May be well-circumscribed.
*+/-Periosteal reaction.
*+/-Sclerotic border.

May be worked-up with angiography to distinguish from a [[vascular malformation]].<ref name=emed1255879dx>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
====Location====
*Usually extremities - femur or proximal tibial.<ref name=emed1255879ov>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>

===Microscopic===
Features:<ref name=emed1255879dx>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
*Hypervascular lesion - '''key diagnostic feature'''.<ref name=enzinger>Enzinger & Weiss's Soft Tissue Tumors. 4th Ed. PP.1007-13. ISBN 0-323-01200-0.</ref>
**Abundant thin-walled branching small vessels of variable size.
***May be described as "[[staghorn vessels]]" or "antler-like" vasculature.
***Cells may "onion-skin" around thin blood vessels.
*Spindle or ovoid shaped cells in nests or sheets.

DDx:
*Other [[vascular tumours]].
*[[Vascular malformations]].
*[[Synovial sarcoma]].
*[[Dermatofibroma]]. (???)

===IHC===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref name=enzinger/>
*Vimentin +ve (usually).
*Desmin -ve (typical).
*Factor VIII -ve (marks endothelium).
*CD34 +ve.
**CD34 usu. -ve in synovial sarcoma.
*CD31 -ve (marks benign endothelium).
*vWF (von Willebrand factor) -ve.

May be in the DDx for [[meningioma]]:<ref>Croul, SE. 8 November 2010.</ref>
*EMA -ve.
*S100 -ve.

===Images===
<gallery>
Image:Neuropathology_case_VI_02.jpg | Anaplastic hemangiopericytoma, low mag. (WC/jensflorian)
Image:Neuropathology_case_VI_03.jpg | Anaplastic hemangiopericytoma, intermed mag. (WC/jensflorian)
Image:Neuropathology_case_VI_04.jpg | Anaplastic hemangiopericytoma, high mag. (WC/jensflorian)
Image:Neuropathology_case_VI_01.jpg | Anaplastic hemangiopericytoma, [[STAT6]] immunostaining. (WC/jensflorian)
</gallery>

=Malignant=
==Low-grade fibromyxoid sarcoma==
*[[AKA]] ''hyalinizing spindle cell tumour''.
*Should '''not''' be confused with ''[[myxofibrosarcoma]]''.
*Abbreviated ''LGFMS''.
{{Main|Low-grade fibromyxoid sarcoma}}

==Adult fibrosarcoma==
*[[AKA]] ''fibrosarcoma''.
*Should '''not''' be confused with [[infantile fibrosarcoma]].

===General===
*Malignant.
*Older adults.
*Locations: head & neck, extremities.

===Microscopic===
Feature:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*Spindle cell lesion.
*[[Herring bone pattern]] - '''key feature'''.
*Mitoses.

DDx (herring bone):
*MPNST.
*Synovial sarcoma.
*Fibrosarcoma.

DDx:
*[[Dermatofibrosarcoma protuberans]] (DFSP) - t(17;22) COLA1/PDGFB.
*[[Congenital-infantile fibrosarcoma]] - t(12;15) ETV6/NTRK3.
*[[Solitary fibrous tumour]].
*[[Synovial sarcoma]] - t(X;18) SYT/SSX.
*[[MPNST]].

Images:
*[http://en.wikipedia.org/wiki/File:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg Herring bone pattern - high mag. (WC)].

===IHC===
Features:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*Vimentin.
*SMA.

==Myxofibrosarcoma==
*Should '''not''' be confused with ''[[low-grade fibromyxoid sarcoma]]''.<ref name=pmid8650138>{{Cite journal | last1 = Mentzel | first1 = T. | last2 = Katenkamp | first2 = D. | last3 = Fletcher | first3 = CD. | title = [Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course]. | journal = Pathologe | volume = 17 | issue = 2 | pages = 116-21 | month = Mar | year = 1996 | doi = | PMID = 8650138 }}</ref>
*[[AKA]] ''myxoid malignant fibrous histiocytoma'' or ''myxoid MFH''.<ref>{{Cite journal | last1 = Fujimura | first1 = T. | last2 = Okuyama | first2 = R. | last3 = Terui | first3 = T. | last4 = Okuno | first4 = K. | last5 = Masu | first5 = A. | last6 = Masu | first6 = T. | last7 = Chiba | first7 = S. | last8 = Kunii | first8 = T. | last9 = Tagami | first9 = H. | title = Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases. | journal = J Cutan Pathol | volume = 32 | issue = 7 | pages = 512-5 | month = Aug | year = 2005 | doi = 10.1111/j.0303-6987.2005.00368.x | PMID = 16008697 }}</ref>
{{Main|Myxofibrosarcoma}}

=See also=
*[[Soft tissue lesions]].

=References=
{{Reflist|2}}

[[Category:Soft tissue lesions]]

Pediatric gastrointestinal pathology

2018-05-06T18:35:41Z

Brigitte: /* Images */

This article deals with '''pediatric gastrointestinal pathology'''. An introduction to pediatric pathology is in the ''[[pediatric pathology]]'' article.

An overview of (adult) gastrointestinal pathology is in the ''[[gastrointestinal pathology]]'' article.

=Birth defects=
==Omphalocele==
===General===
Usually genetic (unlike [[gastroschisis]]) - associated with:<ref name=pmid20809116>{{Cite journal | last1 = Frolov | first1 = P. | last2 = Alali | first2 = J. | last3 = Klein | first3 = MD. | title = Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature. | journal = Pediatr Surg Int | volume = 26 | issue = 12 | pages = 1135-48 | month = Dec | year = 2010 | doi = 10.1007/s00383-010-2701-7 | PMID = 20809116 }}</ref>
*[[Trisomy 18]] (Edwards syndrome) ~ 6% have omphaloceles in a series of 85 cases.<ref name=pmid3191615>{{Cite journal | last1 = Moore | first1 = CA. | last2 = Harmon | first2 = JP. | last3 = Padilla | first3 = LM. | last4 = Castro | first4 = VB. | last5 = Weaver | first5 = DD. | title = Neural tube defects and omphalocele in trisomy 18. | journal = Clin Genet | volume = 34 | issue = 2 | pages = 98-103 | month = Aug | year = 1988 | doi = | PMID = 3191615 }}</ref>
*[[Beckwith-Wiedemann syndrome]].

Presentation:
*Increased AFP.

===Gross===
*Bowel outside of abdomen - covered by membrane/in a sac.

Image:
*[http://library.med.utah.edu/WebPath/PEDHTML/PED006.html Omphalocele (utah.edu)].

==Gastroschisis==
===General===
*Defect considered to be more severe than [[omphalocele]].
*Usually sporadic.

===Gross===
*Bowel outside of abdomen - individual loops of bowel are seen.

Image:
*[http://med.brown.edu/pedisurg/Brown/IBImages/AbdWallDefects/Gastroschisis%202.html Gastroschisis (brown.edu)].

=Luminal pathology=
==Esophageal atresia==
===General===
*Multifactoral.
*Often associated with other abnormalities.

Forms:<ref name=pmid17498283>{{Cite journal | last1 = Spitz | first1 = L. | title = Oesophageal atresia. | journal = Orphanet J Rare Dis | volume = 2 | issue = | pages = 24 | month = | year = 2007 | doi = 10.1186/1750-1172-2-24 | PMID = 17498283 }}</ref>
#Esophageal atresia with distal tracheoesophageal fistula - most common.
#Esophageal atresia without a fistula.

Note:
*The "H-type" tracheoeosphageal fistula is often lumped together with ''esophageal atresia''.<ref name=pmid17498283/>

Image:
*[http://commons.wikimedia.org/wiki/File:Atrezja.jpg Esophageal atresia (WC)].

==Abetalipoproteinemia==
*[[AKA]] ''Bassen-Kornzweig syndrome''.

===General===
*Rare genetic disorder.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/200100 http://www.ncbi.nlm.nih.gov/omim/200100]. Accessed on: 6 April 2011.</ref><ref>{{cite journal |author=Bassen FA, Kornzweig AL |title=Malformation of the erythrocytes in a case of atypical retinitis pigmentosa |journal=Blood |volume=5 |issue=4 |pages=381–87 |year=1950 |month=April |pmid=15411425 |doi= |url=}}</ref>
*GI-related symptoms similar to [[celiac disease]] - malabsorption.

Clinical features:<ref name=pmid24139731>{{Cite journal | last1 = Hammer | first1 = MB. | last2 = El Euch-Fayache | first2 = G. | last3 = Nehdi | first3 = H. | last4 = Feki | first4 = M. | last5 = Maamouri-Hicheri | first5 = W. | last6 = Hentati | first6 = F. | last7 = Amouri | first7 = R. | title = Clinical features and molecular genetics of two Tunisian families with abetalipoproteinemia. | journal = J Clin Neurosci | volume = 21 | issue = 2 | pages = 311-5 | month = Feb | year = 2014 | doi = 10.1016/j.jocn.2013.04.016 | PMID = 24139731 }}</ref>
*Failure to thrive.
*Pigmented retinopathy.

Blood work:<ref name=pmid24139731/>
*Cholesterol - low.
*Triglyceride - low.
*Apolipoprotein B - very low.

===Microscopic===
Features:
*Enterocytes have clear cytoplasm (due to lipid accumulation).

Notes:
*Have abnormal erythrocytes with a spiculated cell membranes ''acanthocyte'' - seen on blood films.

====Images====
<gallery>
Image:Abetalipoproteinemia_-_intermed_mag.jpg | Abetalipoproteinemia - intermed. mag. (WC)
Image:Abetalipoproteinemia_-_very_high_mag.jpg | Abetalipoproteinemia - very high mag. (WC)
</gallery>

==Microvillous inclusion disease==
*[[AKA]] ''Davidson disease''.
===General===
*Autosomal recessive inherited condition - due to mutation in ''MYO5B''.<ref name=pmid18724368>{{cite journal |author=Müller T, Hess MW, Schiefermeier N, ''et al.'' |title=MYO5B mutations cause microvillus inclusion disease and disrupt epithelial cell polarity |journal=Nat. Genet. |volume=40 |issue=10 |pages=1163–5 |year=2008 |month=October |pmid=18724368 |doi=10.1038/ng.225 |url=}}</ref>

===Microscopic===
Features:
*Flat mucosa; no villi.

Notes:
*Appearance similar to [[celiac sprue]]; however, usually lacks the intraepithelial lymphocytic infiltration characteristic of [[celiac sprue]].

Images:
*[http://path.upmc.edu/cases/case163.html Microvillous inclusion disease - several images (upmc.edu)].

===IHC===
*[[Carcinoembryonic antigen]] (CEA) +ve.<ref name=Ref_Sternberg4>{{Ref Sternberg4|}}</ref>

===EM===
*Diagnosis is dependent on [[electron microscopy]].<ref name=pmid11251929>{{cite journal |author=Kennea N, Norbury R, Anderson G, Tekay A |title=Congenital microvillous inclusion disease presenting as antenatal bowel obstruction |journal=Ultrasound Obstet Gynecol |volume=17 |issue=2 |pages=172–4 |year=2001 |pmid=11251929 |doi=10.1046/j.1469-0705.2001.00211.x}}</ref>

Images:
*[http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=1398 MID (gfmer.ch)].

==Cystic fibrosis==
{{Main|Cystic fibrosis}}

===General===
*Genetic.
*May lead to [[meconium ileus]].

===Microscopic===
Features - large bowel:<ref name=pmid710839>{{cite journal |author=Neutra MR, Trier JS |title=Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture |journal=Gastroenterology |volume=75 |issue=4 |pages=701–10 |year=1978 |month=October |pmid=710839 |doi= |url=}}</ref>
*Crypt enlargement.

Notes:
*''Not'' intracellular and extracellular accumulation of mucus. (?)

==Colonic aganglionosis==
*[[AKA]] ''Hirschsprung disease''.
===General===
*Genetic disorder:<ref>{{OMIM|142623}}</ref>
**5-10% familial; RET gene most commonly mutated.
**Several genes involved.
**Inheritance pattern variable.
*Treatment: surgery (''Swenson's procedure'' or ''Duhamel procedure'').<ref name=pmid6649901>{{Cite journal | last1 = Okasora | first1 = T. | last2 = Okamoto | first2 = E. | last3 = Kuwata | first3 = K. | last4 = Toyosaka | first4 = A. | last5 = Ohashi | first5 = S. | last6 = Ueki | first6 = S. | title = Serum and erythrocyte acetylcholine esterase in Hirschsprung's disease. | journal = Z Kinderchir | volume = 38 | issue = 5 | pages = 298-300 | month = Oct | year = 1983 | doi = 10.1055/s-2008-1059992 | PMID = 6649901 }}</ref>

Pathology:
*Failure of neural crest cell migration
**Parasympathetic ganglion cells in submucosal plexus (Meissner plexus) and myenteric plexus (Auerbach plexus) - absent.<ref name=pmid17139897>{{Cite journal | last1 = Vorobyov | first1 = GI. | last2 = Achkasov | first2 = SI. | last3 = Biryukov | first3 = OM. | title = Hirschsprung's disease in adults. | journal = Acta Chir Iugosl | volume = 53 | issue = 2 | pages = 113-6 | month = | year = 2006 | doi = | PMID = 17139897 }}</ref>

Notes:
*Most common reason for litigation in paediatric pathology.<ref>Taylor, G. 19 January 2011.</ref>

===Gross===
Features:
*Dilated bowel; stuffed sausage-look.

Classification:
*Short-segment (75-80%): Rectum, distal sigmoid
*Long-segment HD (10-20%): Beyond splenic flexure
*Total colonic aganglionosis (5-15%): Entire colon.<ref name=pmid25395999>{{Cite web | last = | first = | title = Diagnosis of Hirschsprung's disease with particular emphasis on histopathology. A systematic review of current literature | url = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/ | publisher = | date = | accessdate = 19 August 2017 }}</ref>

**[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/figure/F0001/ Classification of Hirschsprung's disease according to the aganglionic segment length (https://www.ncbi.nlm.nih.gov)]

Image:
*[http://www.pathology.pitt.edu/lectures/gi/colon-a/03.htm Hirschsprung disease (pathology.pitt.edu)].

===Microscopic===
Features:
*Ganglion cells missing in submucosal plexus and myenteric plexus.
**Increasing ganglia proximally into transition zone
*Hypertrophy of neural plexuses.
**Many nerve trunks > 40 μm
*Abnormal submucosal blood vessels may be seen
*+/-Submucosal fibrosis.

Image:
*[http://pathology.mc.duke.edu/research/Histo_course/myent_plexus.jpg Normal myenteric plexus (duke.edu)].<ref>URL: [http://pathology.mc.duke.edu/research/PTH225.html http://pathology.mc.duke.edu/research/PTH225.html]. Accessed on: 11 January 2011.</ref>

===Stains===
*Acetylcholinesterase - marks the abundant, disorganized, nerve fibers.

Image:
*[http://commons.wikimedia.org/wiki/File:Hirschsprung_acetylcholine.jpg Hirschsprung - acetylcholinesterase (WC)].

===IHC===
Features:<ref name=pmid1640323>{{Cite journal | last1 = Luider | first1 = TM. | last2 = van Dommelen | first2 = MW. | last3 = Tibboel | first3 = D. | last4 = Meijers | first4 = JH. | last5 = Ten Kate | first5 = FJ. | last6 = Trojanowski | first6 = JQ. | last7 = Molenaar | first7 = JC. | title = Differences in phosphorylation state of neurofilament proteins in ganglionic and aganglionic bowel segments of children with Hirschsprung's disease. | journal = J Pediatr Surg | volume = 27 | issue = 7 | pages = 815-9 | month = Jul | year = 1992 | doi = | PMID = 1640323 }}</ref>
*NF-M (neurofilament middle) - highlight hypertrophic nerve fascicules
*NF-H (neurofilament high) - highlight hypertrophic nerve fascicules.
*Tau ~ highlights ganglion cells (which are absent in segments affected by Hirschsprung).<ref name=pmid8229560>{{Cite journal | last1 = Deguchi | first1 = E. | last2 = Iwai | first2 = N. | last3 = Goto | first3 = Y. | last4 = Yanagihara | first4 = J. | last5 = Fushiki | first5 = S. | title = An immunohistochemical study of neurofilament and microtubule-associated Tau protein in the enteric innervation in Hirschsprung's disease. | journal = J Pediatr Surg | volume = 28 | issue = 7 | pages = 886-90 | month = Jul | year = 1993 | doi = | PMID = 8229560 }}</ref>
**Nerve fibres +ve control.

Others<ref>{{Cite book | last1 = Lin | first1 = Fan | last2 = Prichard | first2 = Jeffrey | title = Handbook of practical immunohistochemistry : frequently asked question | date = | publisher = | location = | isbn = 9781493915774 | pages = }}</ref>
:
*Calretinin ~ -ve, Usually negative in hypertrophied nerve fibers in Hirschsprung’s disease, superior to acetylcholinesterase.
*NSE ~ -ve, Highlights ganglion cells to exclude Hirschsprung’s disease; specific but not very sensitive.

==Meconium ileus==
===General===
*Classically due to ''[[cystic fibrosis]]''.
*May lead to ''[[meconium peritonitis]]''.
*Can be mimicked by [[CMV]] infection.<ref>{{cite journal |author=Déchelotte PJ, Mulliez NM, Bouvier RJ, Vanlieféringhen PC, Lémery DJ |title=Pseudo-meconium ileus due to cytomegalovirus infection: a report of three cases |journal=Pediatr Pathol |volume=12 |issue=1 |pages=73–82 |year=1992 |pmid=1313975 |doi= |url=}}</ref>

===Gross===
Features:
*Thick.
**High viscosity.
*Green.

====Image====
*[http://library.med.utah.edu/WebPath/jpeg3/PERI175.jpg Meconium ileus (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html]. Accessed on: 3 December 2011.</ref>

===Microscopic===
Features:
*Meconium-laden macrophages. (???)

==Meconium peritonitis==
===General===
*May be due to a number of causes:
**Aganglionosis ([[Hirschsprung disease]]).
**[[Meconium ileus]].

===Microscopic===
Features:
*Brown granular material - '''key feature'''.
*+/-Multinucleated giant cells.
*Inflammatory infiltrate (PMNs, lymphocytes, plasma cells).

Image:
*[http://www.pathologyoutlines.com/caseofweek/case2008106image2.jpg Meconium peritonitis - gross (pathologyoutlines.com)].

==Necrotizing enterocolitis==
*Abbreviated ''NEC''.
===General===
*Disease primarily of premature babies.
*Diagnosed by imaging.

Note:
*''Enterocolitis'' = inflammation of [[small bowel]] and [[colon]].<ref>URL: [http://medical-dictionary.thefreedictionary.com/enterocolitis http://medical-dictionary.thefreedictionary.com/enterocolitis]. Accessed on: 10 October 2011.</ref>
**''Necrotizing enteritis'' = small bowel only.

===Microscopic===
Features:
*Large spaces.

DDx:
*[[Pneumatosis intestinalis]].

Images:
*[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)].
*[https://library.med.utah.edu/WebPath/PEDHTML/PED045.html NEC - micro].

==Autoimmune enteropathy==
{{Main|Autoimmune enteropathy}}

=Pancreas=
{{Main|Pancreas}}
==Pancreatic islet cell hyperplasia==
===General===
*Associated with maternal [[diabetes]].

===Microscopic===
Features:
*Marked size variation of pancreatic islets.
**Normal islets ~ 150 micrometers (diameter). Hyperplastic islets - up to ~500 micrometers (diameter).

Image:
*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].

=Liver=
{{Main|Liver pathology}}
==Giant cell hepatitis==
*[[AKA]] ''neonatal giant cell hepatitis'', abbreviated ''NGCH''.
===General===
*Rare.

Etiology:
*Unknown - possibly viral, autoimmune and/or drugs.<ref name=pmid21043007>{{Cite journal | last1 = Hartl | first1 = J. | last2 = Buettner | first2 = R. | last3 = Rockmann | first3 = F. | last4 = Farkas | first4 = S. | last5 = Holstege | first5 = A. | last6 = Vogel | first6 = C. | last7 = Schnitzbauer | first7 = A. | last8 = Schlitt | first8 = HJ. | last9 = Schoelmerich | first9 = J. | title = Giant cell hepatitis: an unusual cause of fulminant liver failure. | journal = Z Gastroenterol | volume = 48 | issue = 11 | pages = 1293-6 | month = Nov | year = 2010 | doi = 10.1055/s-0029-1245476 | PMID = 21043007 }}</ref>
*One large series suggests that, in the neonatal population, with follow-up the causes are:
**~49% idiopathic.
**~16% pan-hypopituitarism.
**~8% biliary atresia.
**~6% Alagille syndrome
**~6% bile salt defects.

Notes:
*May be seen in adults.<ref name=pmid21325763>{{Cite journal | last1 = Hayashi | first1 = H. | last2 = Narita | first2 = R. | last3 = Hiura | first3 = M. | last4 = Abe | first4 = S. | last5 = Tabaru | first5 = A. | last6 = Tanimoto | first6 = A. | last7 = Sasaguri | first7 = Y. | last8 = Harada | first8 = M. | title = A case of adult autoimmune hepatitis with histological features of giant cell hepatitis. | journal = Intern Med | volume = 50 | issue = 4 | pages = 315-9 | month = | year = 2011 | doi = | PMID = 21325763 }}</ref>
*Reported association with [[subdural hematoma]].<ref name=pmid21331818>{{Cite journal | last1 = Guddat | first1 = SS. | last2 = Ehrlich | first2 = E. | last3 = Martin | first3 = H. | last4 = Tsokos | first4 = M. | title = Fatal spontaneous subdural bleeding due to neonatal giant cell hepatitis: a rare differential diagnosis of shaken baby syndrome. | journal = Forensic Sci Med Pathol | volume = 7 | issue = 3 | pages = 294-7 | month = Sep | year = 2011 | doi = 10.1007/s12024-011-9227-8 | PMID = 21331818 }}</ref>

===Microscopic===
Features:<ref name=pmid20871223>{{Cite journal | last1 = Torbenson | first1 = M. | last2 = Hart | first2 = J. | last3 = Westerhoff | first3 = M. | last4 = Azzam | first4 = RK. | last5 = Elgendi | first5 = A. | last6 = Mziray-Andrew | first6 = HC. | last7 = Kim | first7 = GE. | last8 = Scheimann | first8 = A. | title = Neonatal giant cell hepatitis: histological and etiological findings. | journal = Am J Surg Pathol | volume = 34 | issue = 10 | pages = 1498-503 | month = Oct | year = 2010 | doi = 10.1097/PAS.0b013e3181f069ab | PMID = 20871223 }}</ref>
*Giant hepatocytes with multiple nuclei - '''key feature'''.
**Typically ~35% of hepatocytes affected.
*Minimal or absent inflammation portal and lobular inflammation.
*Lobular cholestasis.

====Images====

==Biliary atresia==
===General===
*1/3 of neonatal cholestasis.<ref name=Ref_PCPBoD8|464>{{Ref PCPBoD8|464}}</ref>
*Etiology - various.
**Viral - possibly rotavirus.<ref name=pmid22123643>{{Cite journal | last1 = Hertel | first1 = PM. | last2 = Estes | first2 = MK. | title = Rotavirus and biliary atresia: can causation be proven? | journal = Curr Opin Gastroenterol | volume = 28 | issue = 1 | pages = 10-7 | month = Jan | year = 2012 | doi = 10.1097/MOG.0b013e32834c7ae4 | PMID = 22123643 }}</ref>
**Genetic/syndromic - several.<ref name=pmid20425482>{{Cite journal | last1 = Santos | first1 = JL. | last2 = Choquette | first2 = M. | last3 = Bezerra | first3 = JA. | title = Cholestatic liver disease in children. | journal = Curr Gastroenterol Rep | volume = 12 | issue = 1 | pages = 30-9 | month = Feb | year = 2010 | doi = 10.1007/s11894-009-0081-8 | PMID = 20425482 }}</ref>

===Microscopic===
Features:<ref name=Ref_PCPBoD8|464/>
*Bile duct proliferation.
*Portal tract edema.
*Portal tract fibrosis.

Image:
*[http://oac.med.jhmi.edu/pathconcepts/ShowImage.cfm?TutorialID=3&ConceptID=12&ImageID=244 Biliary atresia (jhmi.edu)].

=See also=
*[[Pediatric pathology]].
*[[Gastrointestinal pathology]].

=References=
{{Reflist|2}}

[[Category:Pediatric pathology]]

Colitis cystica profunda

2018-05-05T17:11:58Z

Brigitte: /* Microscopic */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image =
| Width =
| Caption =
| Micro = mucin filled cysts in submucosa or deeper
| Subtypes =
| LMDDx = well-differentiated [[colorectal adenocarcinoma]], [[mucinous adenocarcinoma]]
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = [[colon]] and [[rectum]], usu. distal
| Assdx = [[cap polyposis]]
| Syndromes =
| Clinicalhx =
| Signs =
| Symptoms =
| Prevalence = very rare (case reports)
| Bloodwork =
| Rads =
| Endoscopy = polypoid lesion(s)
| Prognosis = good, benign
| Other =
| ClinDDx = other [[GI polyps|polypoid lesions]]
}}
'''Colitis cystica profunda''', abbreviated '''CCP''', a very rare [[gastrointestinal pathology]].

==General==
*Very rare ~ 200 cases reported.<ref name=pmid17891698>{{Cite journal | last1 = Dolar | first1 = E. | last2 = Kiyici | first2 = M. | last3 = Yilmazlar | first3 = T. | last4 = Gürel | first4 = S. | last5 = Nak | first5 = SG. | last6 = Gülten | first6 = M. | title = Colitis cystica profunda. | journal = Turk J Gastroenterol | volume = 18 | issue = 3 | pages = 206-7 | month = Sep | year = 2007 | doi = | PMID = 17891698 }}</ref>
*Benign - may mimic [[adenocarcinoma]].<ref name=pmid16018526/>
**Has been reported in conjunction with [[colonic adenocarcinoma]].<ref>{{Cite journal | last1 = Mitsunaga | first1 = M. | last2 = Izumi | first2 = M. | last3 = Uchiyama | first3 = T. | last4 = Sawabe | first4 = A. | last5 = Tanida | first5 = E. | last6 = Hosono | first6 = K. | last7 = Abe | first7 = T. | last8 = Shirahama | first8 = K. | last9 = Kanesaki | first9 = A. | title = Colonic adenocarcinoma associated with colitis cystica profunda. | journal = Gastrointest Endosc | volume = 69 | issue = 3 Pt 2 | pages = 759-60; discussion 760-1 | month = Mar | year = 2009 | doi = 10.1016/j.gie.2008.12.240 | PMID = 19251024 }}</ref>
*The same process in the [[small bowel]] is ''enteritis cystica profunda''.<ref name=pmid17891698/>
*Association with [[cap polyposis]].<ref name=pmid24118052>{{Cite journal | last1 = Arana | first1 = R. | last2 = Fléjou | first2 = JF. | last3 = Parc | first3 = Y. | last4 = El-Murr | first4 = N. | last5 = Cosnes | first5 = J. | last6 = Svrcek | first6 = M. | title = Cap polyposis and colitis cystica profunda: a rare association. | journal = Histopathology | volume = | issue = | pages = | month = Sep | year = 2013 | doi = 10.1111/his.12292 | PMID = 24118052 }}</ref>
==Gross==
Features:<ref name=pmid18563984>{{Cite journal | last1 = Higuera Alvarez | first1 = R. | last2 = García | first2 = Jde L. | last3 = San Miguel | first3 = G. | last4 = Castro | first4 = B. | title = [Colitis cystica profunda]. | journal = Rev Esp Enferm Dig | volume = 100 | issue = 4 | pages = 240-2 | month = Apr | year = 2008 | doi = | PMID = 18563984 }}</ref>
*Colon and rectum - usu. distal location.
*Typically focal.
*[[GI polyps|Polypoid lesions]].

==Microscopic==
Features:
*Mucin filled cysts in the submucosa<ref name=pmid16018526/><ref name=pmid18563984/> or deeper.<ref name=pmid21212765/>
*+/-Inflammation.

DDx:
*Well-differentiated [[colorectal adenocarcinoma]].<ref name=pmid16018526>{{Cite journal | last1 = Kayaçetin | first1 = E. | last2 = Kayaçetin | first2 = S. | title = Colitis cystica profunda simulating rectal carcinoma. | journal = Acta Chir Belg | volume = 105 | issue = 3 | pages = 306-8 | month = | year = | doi = | PMID = 16018526 }}</ref>
*Metastatic [[mucinous adenocarcinoma]].

Image:

==See also==
*[[Colon]].
*[[Rectum]].
*[[Pneumatosis cystoides intestinalis]].
*[[Pneumatosis intestinalis]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Gastrointestinal pathology]]

Dermatologic neoplasms

2018-05-03T14:33:23Z

Brigitte: /* Microscopic */

This article deals with '''dermatologic neoplasms''', also known as '''skin tumours'''. It includes '''dermatologic cancer''', which can be deadly. Collectively, dermatologic cancers are the most common form of cancer.

An introduction to dermatopathy is found in the ''[[dermatopathology]]'' article. Non-malignant disease is covered in the ''[[non-malignant skin disease]]'' article.

=The Big Three malignant=
==Basal cell carcinoma==
{{Main|Basal cell carcinoma}}

==Squamous cell carcinoma of the skin==
*Abbreviated ''skin SCC'', ''SCC of the skin'', and ''SCC of skin''.
{{Main|Squamous cell carcinoma of the skin}}

==Melanoma==
{{Main|Malignant melanoma}}
*Known as the great mimicker in pathology; it may look like many things.

=Less common malignant=
==Dermatofibrosarcoma protuberans==
*Abbreviated ''DFSP''.
{{Main|Dermatofibrosarcoma protuberans}}

==Cutaneous B-cell lymphoma==
*Abbreviated CBCL.

===General===
*CBCL is less common than cutaneous T-cell lymphoma (CTCL).<ref>URL: [http://emedicine.medscape.com/article/1099540-overview http://emedicine.medscape.com/article/1099540-overview]. Accessed on: 24 August 2010.</ref>

===Microscopic===
Features:
*Dermal lymphoid infiltrate.
*"Grenz zone" - space between the epidermis and the dermal infiltrate - '''key feature'''.

===IHC===
*B cell and T cell markers.

==Cutaneous T-cell lymphoma==
*Abbreviated CTCL.
{{Main|Cutaneous T-cell lymphoma}}

==Merkel cell carcinoma==
{{Main|Merkel cell carcinoma}}

==Eccrine carcinoma==
===General===
*Arises from the proximal sweat duct.

===Microscopic===
Features:
*Pleomorphic nuclei with nucleoli.
*Duct-like structures - '''key feature'''.
*Extends from dermis into epidermis (follows path of a benign sweat duct).

Notes:
*May resemble [[Extramammary Paget's disease]]/[[Paget's disease of the breast]].

==Kaposi sarcoma==
:See ''[[Kaposi sarcoma]]''.

==Sebaceous carcinoma==
{{Main|Sebaceous carcinoma}}

==Microcystic adnexal carcinoma==
{{Main|Microcystic adnexal carcinoma}}

==Trichilemmal carcinoma==
{{Main|Trichilemmal carcinoma}}

==Lymphomatoid papulosis==
===General===
*Rare.
*Benign behaviour.

===Microscopic===
Features:
*Dermal lymphocytosis.
**No epidermal lymphocytes.
*Focal nuclear atypia.

DDx:
*[[CTCL]].
*Cutaneous [[ALCL]].

===IHC===
*CD30 +ve.<ref>URL: [http://path.upmc.edu/cases/case513/dx.html http://path.upmc.edu/cases/case513/dx.html]. Accessed on: 25 January 2012.</ref>

=Rare malignant=
==Basosquamous carcinoma==
:Should '''not''' be confused with ''basaloid [[squamous cell carcinoma]]'' ([[AKA]] ''squamous cell carcinoma, basaloid variant'').
===General===
*Very rare.
**Largest case series, as of 2000, 35 cases.<ref name=pmid10717618>{{Cite journal | last1 = Martin | first1 = RC. | last2 = Edwards | first2 = MJ. | last3 = Cawte | first3 = TG. | last4 = Sewell | first4 = CL. | last5 = McMasters | first5 = KM. | title = Basosquamous carcinoma: analysis of prognostic factors influencing recurrence. | journal = Cancer | volume = 88 | issue = 6 | pages = 1365-9 | month = Mar | year = 2000 | doi = | PMID = 10717618 }}
</ref>
*May be considered an aggressive variant of [[basal cell carcinoma]].<ref name=pmid10717618/>
*Aggressive behaviour.<ref name=pmid12859383>{{Cite journal | last1 = Bowman | first1 = PH. | last2 = Ratz | first2 = JL. | last3 = Knoepp | first3 = TG. | last4 = Barnes | first4 = CJ. | last5 = Finley | first5 = EM. | title = Basosquamous carcinoma. | journal = Dermatol Surg | volume = 29 | issue = 8 | pages = 830-2; discussion 833 | month = Aug | year = 2003 | doi = | PMID = 12859383 }}.</ref>

===Microscopic===
Features:
*Has features of both [[basal cell carcinoma]] and [[squamous cell carcinoma of the skin|squamous cell carcinoma]].<ref name=pmid12859383/>
**BCC component usually predominant.<ref name=Ref_Derm397>{{Ref Derm|397}}</ref>

Note:
*''Busam'' notes that there is disagreement about what defines this tumour;<ref name=Ref_Derm372>{{Ref Derm|372}}</ref> however, he goes on the describe it as a ''[[collision tumour]]''.<ref name=Ref_Derm397>{{Ref Derm|397}}</ref>

DDx:
*Basaloid [[squamous cell carcinoma]].
*[[Basal cell carcinoma]] with squamous differentiation.

=Intermediate=
==Atypical fibroxanthoma==
*Abbreviated ''AFX''.
{{Main|Atypical fibroxanthoma}}

=Benign=
==Syringoma==
===General===
*Benign sweat duct tumour.
*Eccrine differentiation.
*Usually close to lower [[eyelid]].<ref>{{Ref PBoD8|1177}}</ref>

===Microscopic===
Features:<ref>URL: [http://emedicine.medscape.com/article/1059871-diagnosis http://emedicine.medscape.com/article/1059871-diagnosis]. Accessed on: 12 May 2010.</ref>
*Proliferation of benign ducts with lined by a bilayer (as in normal sweat ducts) with abnormal architecture:
**Tadpole like appearing ducts.

DDx:
*Syringomatous adenomas of nipple (AKA syringoma of the nipple).<ref name=pmid22355740>{{Cite journal | last1 = Boecker | first1 = W. | last2 = Junkers | first2 = T. | last3 = Reusch | first3 = M. | last4 = Buerger | first4 = H. | last5 = Korsching | first5 = E. | last6 = Metze | first6 = D. | last7 = Decker | first7 = T. | last8 = Loening | first8 = T. | last9 = Lange | first9 = A. | title = Origin and differentiation of breast nipple syringoma. | journal = Sci Rep | volume = 2 | issue = | pages = 226 | month = | year = 2012 | doi = 10.1038/srep00226 | PMID = 22355740 |URL = http://www.nature.com/srep/2012/120117/srep00226/full/srep00226.html }}</ref>
*[[Chondroid syringoma]]. (???)

Images:
*[http://www.flickr.com/photos/euthman/2329061316/ Syringoma (flickr.com)].
*[http://dermatology.cdlib.org/144/tumors/axillary_syringoma/2.jpg Syringoma (dermatology.cdlib.org)].<ref>{{Cite journal | last1 = Nosrati | first1 = N. | last2 = Coleman | first2 = NM. | last3 = Hsu | first3 = S. | title = Axillary syringomas. | journal = Dermatol Online J | volume = 14 | issue = 4 | pages = 13 | month = | year = 2008 | doi = | PMID = 18627735 |URL = http://dermatology.cdlib.org/144/tumors/axillary_syringoma/hsu.html}}</ref>

==Chondroid syringoma==
*Used to be called ''mixed tumour of skin''.<ref name=pmid19693940>{{Cite journal | last1 = Kumar | first1 = B. | title = Chondroid syringoma diagnosed by fine needle aspiration cytology. | journal = Diagn Cytopathol | volume = 38 | issue = 1 | pages = 38-40 | month = Jan | year = 2010 | doi = 10.1002/dc.21159 | PMID = 19693940 }}</ref>

===General===
*Mixed apocrine & eccrine tumour of skin, usually in the head & neck<ref name=pmid19693940/>, especially nose and cheek.<ref name=pmid19633639/>
*May be in major and minor salivary glands.<ref name=pmid19633639>{{Cite journal | last1 = Rauso | first1 = R. | last2 = Santagata | first2 = M. | last3 = Tartaro | first3 = G. | last4 = Filipi | first4 = M. | last5 = Colella | first5 = G. | title = Chondroid syringoma: a rare tumor of orofacial region. | journal = Minerva Stomatol | volume = 58 | issue = 7-8 | pages = 383-8 | month = | year = | doi = | PMID = 19633639 }}</ref>

===Microscopic===
Features:
*Mix tumour with:<ref name=pmid19693940/>
*#Epithelial component:
*#*Nests of cells with:
*#**Moderate dull eosinophilic cytoplasm.
*#**Round/ovoid nuclei with nucleoli.
*#Mesenchymal component - '''key feature''':
*#*[[Chondromyxoid stroma]].

Images:
*[https://www.dermnetnz.org/topics/apocrine-mixed-tumour-pathology Chondroid syringoma (DermnetNZ)].

==Dermal cylindroma==
{{Main|Dermal cylindroma}}

==Keratoacanthoma==
{{Main|Keratoacanthoma}}

==Sebaceous adenoma==
===General===
*Seen in [[Muir-Torre syndrome]] - a variant of [[Lynch syndrome]] (hereditary non-polyposis colon cancer).

Notes:
*Sebaceous lesions (from benign to malignant): [[sebaceous hyperplasia]], sebaceous adenoma, sebaceoma, [[sebaceous carcinoma]].

===Microscopic===
Features:
*Abnormal sebaceous glands (pale fluffy cytoplasm):
**Increased basal epithelium.
**Multiple dilated glands - opening to the surface.

====Images====
<gallery>
Image:Sebaceous_adenoma_-_low_mag.jpg | Sebaceous adenoma - low mag. (WC/Nephron)
Image:Sebaceous_adenoma_-_high_mag.jpg | Sebaceous adenoma - high mag. (WC/Nephron)
</gallery>
www:
*[http://dermatlas.med.jhmi.edu/derm/indexDisplay.cfm?ImageID=587283984 Sebaceous adenoma (jhmi.edu)].

==Trichilemmoma==
*May be spelled ''tricholemmoma''.
{{Main|Trichilemmoma}}

==Eccrine poroma==
===General===
*Benign tumour arising from the distal sweat duct.
*Erythematous - gross.

===Microscopic===
Features:<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675(06)70190-5 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675(06)70190-5]. Accessed on: 2 July 2010.</ref>
*Broad sheets of basaloid cells - attached to the epidermis - containing ductal structures - '''key feature'''.
*Biphasic stroma:
*#Edematous stroma.
*#Sclerotic stroma.
*Moderate nuclear pleomorphism.
*+/-Occasional mitoses.

Notes:
*Area above gland appears crusted.

DDx:
*[[Trichilemmoma]].
*[[Nodular hidradenoma]].

====Images====
<gallery>
Image: SkinTumors-P7150495.JPG | EP. (WC)
</gallery>
www:
*[http://www.flickr.com/photos/40981620@N04/3808316834/in/photostream/ Eccrine poroma - low mag. (flickr.com)]
*[http://www.flickr.com/photos/40981620@N04/3807502071/in/photostream Eccrine poroma - intermed. mag. (flickr.com)].

==Nodular hidradenoma==
*[[AKA]] ''eccrine acrospiroma''.<ref name=pmid18319032>{{Cite journal | last1 = Punia | first1 = RP. | last2 = Garg | first2 = S. | last3 = Bal | first3 = A. | last4 = Mohan | first4 = H. | title = Pigmented nodular hidradenoma masquerading as nodular malignant melanoma. | journal = Dermatol Online J | volume = 14 | issue = 1 | pages = 15 | month = | year = 2008 | doi = | PMID = 18319032 |URL = http://dermatology.cdlib.org/141/case_presentations/hidradenoma/punia.html }}</ref>
{{Main|Nodular hidradenoma}}

==Trichoblastoma==
{{Main|Trichoblastoma}}

==Trichofolliculoma==
{{Main|Trichofolliculoma}}

==Apocrine carcinoma of the skin==
===General===
*Rare.<ref name=pmid7678545>{{Cite journal | last1 = Paties | first1 = C. | last2 = Taccagni | first2 = GL. | last3 = Papotti | first3 = M. | last4 = Valente | first4 = G. | last5 = Zangrandi | first5 = A. | last6 = Aloi | first6 = F. | title = Apocrine carcinoma of the skin. A clinicopathologic, immunocytochemical, and ultrastructural study. | journal = Cancer | volume = 71 | issue = 2 | pages = 375-81 | month = Jan | year = 1993 | doi = | PMID = 7678545 }}</ref>
*Usually very good prognosis.<ref name=pmid7678545/>

===Microscopic===
Features:<ref name=pmid7678545/>
*Nests.
*Apocrine snouts - "decapitation secretion"

DDx:
*[[Paget disease of the breast]]/[[Extramammary Paget disease]].

====Images====
<gallery>
Image:Apocrine_carcinoma_-_intermed_mag.jpg | Apocrine carcinoma - intermed. mag. (WC/Nephron)
Image:Apocrine_carcinoma_-_high_mag.jpg | Apocrine carcinoma - high mag. (WC/Nephron)
</gallery>
===Stains===
Features:<ref name=pmid7678545/>
*PAS +ve.
*PASD +ve.

===IHC===
*[[GCDFP-15]] (gross cystic disease fluid protein-15) +ve.<ref name=pmid7678545/>

==Dermatomyofibroma==
:Should ''not'' be confused with [[dermatofibroma]].
*Abbreviated ''DMF''.
===General===
*Uncommon.

===Microscopic===
Features:<ref name=Ref_Derm504>{{Ref Derm|504}}</ref>
*Poorly formed fasicles parallel to the skin surface, usu. restricted to the superficial dermis.
*Moderate cellular density - less cellular than [[DFSP]].
*Eosinophilic cytoplasm.

DDx:
*[[DFSP]].
*[[Dermatofibroma]].

Images:
*[http://www.dermpedia.org/node/8822 DMF - low mag. (dermpedia.org)].
*[http://www.dermpedia.org/node/8824 DMF - high mag. (dermpedia.org)].

===IHC===
Features:<ref name=Ref_Derm504>{{Ref Derm|504}}</ref>
*CD10 +ve.
*Vimentin +ve.

Others:<ref name=Ref_Derm504>{{Ref Derm|504}}</ref>
*CD34 -ve.
*Factor XIIIa -ve.
*S-100 -ve.

==Papillary eccrine adenoma==
*Abbreviated ''[[PEA]]''.
===General===
*Uncommon.
*Benign.<ref name=pmid857729>{{Cite journal | last1 = Rulon | first1 = DB. | last2 = Helwig | first2 = EB. | title = Papillary eccrine adenoma. | journal = Arch Dermatol | volume = 113 | issue = 5 | pages = 596-8 | month = May | year = 1977 | doi = | PMID = 857729 }}</ref>

Treatment:
*Excision.<ref>URL: [http://archderm.jamanetwork.com/article.aspx?articleid=541159 http://archderm.jamanetwork.com/article.aspx?articleid=541159]. Accessed on: 10 December 2012.</ref>
===Gross===
*Central location.

Note:
*The ''digital papillary adenoma'' is considered malignant; the AFIP says these are best classified as ''adenocarcinomas'', i.e. ''[[digital papillary adenocarcinoma]]''.<ref name=pmid10843279>{{Cite journal | last1 = Duke | first1 = WH. | last2 = Sherrod | first2 = TT. | last3 = Lupton | first3 = GP. | title = Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited). | journal = Am J Surg Pathol | volume = 24 | issue = 6 | pages = 775-84 | month = Jun | year = 2000 | doi = | PMID = 10843279 }}</ref>

===Microscopic===
Features:<ref name=pmid17642667>{{Cite journal | last1 = Laxmisha | first1 = C. | last2 = Thappa | first2 = DM. | last3 = Jayanthi | first3 = S. | title = Papillary eccrine adenoma. | journal = Indian J Dermatol Venereol Leprol | volume = 70 | issue = 6 | pages = 370-2 | month = | year = | doi = | PMID = 17642667 | URL = http://www.ijdvl.com/article.asp?issn=0378-6323;year=2004;volume=70;issue=6;spage=370;epage=372;aulast=Laxmisha }}</ref><ref name=pmid9793207/>
*Well-circumscribed lesions consisting of multiple cystic spaces lined by a bilayered epithelium with:
**Papillary projections into the lumen.
**Amorphous eosinophilic material in the cystic spaces.
**Surrounded by a fibrous stroma.<ref name=pmid9508346>{{Cite journal | last1 = Mizuoka | first1 = H. | last2 = Senzaki | first2 = H. | last3 = Shikata | first3 = N. | last4 = Uemura | first4 = Y. | last5 = Tsubura | first5 = A. | title = Papillary eccrine adenoma: immunohistochemical study and literature review. | journal = J Cutan Pathol | volume = 25 | issue = 1 | pages = 59-64 | month = Jan | year = 1998 | doi = | PMID = 9508346 }}</ref>

Note:
*May appear to have more than two cell layers.

DDx:
*[[Digital papillary adenocarcinoma]] - location important.
*[[Tubular apocrine adenoma]] (tubulopapillary hidradenoma<ref name=pmid1566975>{{Cite journal | last1 = Fox | first1 = SB. | last2 = Cotton | first2 = DW. | title = Tubular apocrine adenoma and papillary eccrine adenoma. Entities or unity? | journal = Am J Dermatopathol | volume = 14 | issue = 2 | pages = 149-54 | month = Apr | year = 1992 | doi = | PMID = 1566975 }}</ref>) - a related tumour.<ref name=pmid8238787>{{Cite journal | last1 = Ishiko | first1 = A. | last2 = Shimizu | first2 = H. | last3 = Inamoto | first3 = N. | last4 = Nakmura | first4 = K. | title = Is tubular apocrine adenoma a distinct clinical entity? | journal = Am J Dermatopathol | volume = 15 | issue = 5 | pages = 482-7 | month = Oct | year = 1993 | doi = | PMID = 8238787 }}</ref>

Image:
*[http://www.ijdvl.com/viewimage.asp?img=ijdvl_2004_70_6_370_13482_2.jpg PEA - crappy image (ijdvl.com)].<ref name=pmid17642667/>

===IHC===
Outer layer of epithelium:<ref name=pmid9508346/>
*SMA-alpha +ve.
*Keratin 14 +ve.
Inner layer of epithelium:<ref name=pmid9508346/>
*Keratin 8 +ve.

Other stains:<ref name=pmid9793207>{{Cite journal | last1 = Guccion | first1 = JG. | last2 = Patterson | first2 = RH. | last3 = Nayar | first3 = R. | last4 = Saini | first4 = NB. | title = Papillary eccrine adenoma: an ultrastructural and immunohistochemical study. | journal = Ultrastruct Pathol | volume = 22 | issue = 3 | pages = 263-9 | month = | year = | doi = | PMID = 9793207 }}</ref>
*Vimentin +ve.
*CEA +ve.
*[[EMA]] +ve.
*S-100 +ve.

===Sign out===
<pre>
SKIN LESION, LEFT PARIETAL SCALP, BIOPSY:
- PAPILLARY ECCRINE ADENOMA.
</pre>

====Micro====
The sections show a well-circumscribed multi-locular superficial dermal lesion with a bilayered epithelium and intracystic papillary projections. The cystic spaces contain amorphous eosinophilic material. The cystic component is surrounded by a dense fibrous stroma with a mixed inflammatory infiltrate, consisting primary of plasma cells and lymphocytes.

There is no significant nuclear atypia and no mitotic activity is appreciated. The overlying epidermis matures appropriately. A granular layer is present.

=See also=
*[[Dermatopathology]].
*[[Cytopathology]].

=References=
{{reflist|2}}

[[Category:Dermatopathology]]

Trichofolliculoma

2018-05-03T14:22:17Z

Brigitte: /* Images */

'''Trichofolliculoma''' is an uncommon benign [[dermatologic neoplasm]].

==General==
*Benign.

==Microscopic==
Features:<ref name=Ref_Derm382>{{Ref Derm|382}}</ref>
*Irregular hair follicle (basilar nest of cells with an acellular hair shaft) with:
**Smaller satellites (follicles) consisting of well-circumscribed basilar cells.

Note:
*Lack artificial clefting between the (basilar) nests and stroma (seen in [[BCC]]).
*Surrounding stroma does not have a basophilic tingle (seen in [[BCC]]).

DDx:
*[[Trichoblastoma]].
*[[Basal cell carcinoma]].
*[[Pilar sheath acanthoma]].

===Images===
www:

Trichofolliculoma [https://www.dermnetnz.org/topics/trichofolliculoma-pathology DermNet]
<gallery>
Image:SkinTumors-P6190340.JPG | Trichofolliculoma. (WC)
</gallery>

==See also==
*[[Dermatologic neoplasms]].

==References==
{{Reflist|2}}

[[Category:Dermatologic neoplasms]]
[[Category:Diagnosis]]

Trichofolliculoma

2018-05-03T14:20:20Z

Brigitte: /* Images */

'''Trichofolliculoma''' is an uncommon benign [[dermatologic neoplasm]].

==General==
*Benign.

==Microscopic==
Features:<ref name=Ref_Derm382>{{Ref Derm|382}}</ref>
*Irregular hair follicle (basilar nest of cells with an acellular hair shaft) with:
**Smaller satellites (follicles) consisting of well-circumscribed basilar cells.

Note:
*Lack artificial clefting between the (basilar) nests and stroma (seen in [[BCC]]).
*Surrounding stroma does not have a basophilic tingle (seen in [[BCC]]).

DDx:
*[[Trichoblastoma]].
*[[Basal cell carcinoma]].
*[[Pilar sheath acanthoma]].

===Images===
www:
DermNet [https://www.dermnetnz.org/topics/trichofolliculoma-pathology]
<gallery>
Image:SkinTumors-P6190340.JPG | Trichofolliculoma. (WC)
</gallery>

==See also==
*[[Dermatologic neoplasms]].

==References==
{{Reflist|2}}

[[Category:Dermatologic neoplasms]]
[[Category:Diagnosis]]

Trichofolliculoma

2018-05-03T14:19:50Z

Brigitte: /* Images */

'''Trichofolliculoma''' is an uncommon benign [[dermatologic neoplasm]].

==General==
*Benign.

==Microscopic==
Features:<ref name=Ref_Derm382>{{Ref Derm|382}}</ref>
*Irregular hair follicle (basilar nest of cells with an acellular hair shaft) with:
**Smaller satellites (follicles) consisting of well-circumscribed basilar cells.

Note:
*Lack artificial clefting between the (basilar) nests and stroma (seen in [[BCC]]).
*Surrounding stroma does not have a basophilic tingle (seen in [[BCC]]).

DDx:
*[[Trichoblastoma]].
*[[Basal cell carcinoma]].
*[[Pilar sheath acanthoma]].

===Images===
www:
[https://www.dermnetnz.org/topics/trichofolliculoma-pathology]
<gallery>
Image:SkinTumors-P6190340.JPG | Trichofolliculoma. (WC)
</gallery>

==See also==
*[[Dermatologic neoplasms]].

==References==
{{Reflist|2}}

[[Category:Dermatologic neoplasms]]
[[Category:Diagnosis]]

Trichofolliculoma

2018-05-03T14:19:14Z

Brigitte: /* Images */

'''Trichofolliculoma''' is an uncommon benign [[dermatologic neoplasm]].

==General==
*Benign.

==Microscopic==
Features:<ref name=Ref_Derm382>{{Ref Derm|382}}</ref>
*Irregular hair follicle (basilar nest of cells with an acellular hair shaft) with:
**Smaller satellites (follicles) consisting of well-circumscribed basilar cells.

Note:
*Lack artificial clefting between the (basilar) nests and stroma (seen in [[BCC]]).
*Surrounding stroma does not have a basophilic tingle (seen in [[BCC]]).

DDx:
*[[Trichoblastoma]].
*[[Basal cell carcinoma]].
*[[Pilar sheath acanthoma]].

===Images===
www:

<gallery>
Image:SkinTumors-P6190340.JPG | Trichofolliculoma. (WC)
</gallery>

==See also==
*[[Dermatologic neoplasms]].

==References==
{{Reflist|2}}

[[Category:Dermatologic neoplasms]]
[[Category:Diagnosis]]

Adenoid cystic carcinoma

2018-04-26T13:37:07Z

Brigitte: /* www */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Adenoid cystic carcinoma - high mag.jpg
| Width =
| Caption = Adenoid cystic carcinoma. [[H&E stain]].
| Micro = [[cribriform architecture]] (other patterns: solid, cords, (bilayered) tubules), cystic spaces filled with basophilic material, scant cytoplasm in most cells, nucleus - small, hyaline stroma
| Subtypes =
| LMDDx = [[pleomorphic adenoma]], [[epithelial-myoepithelial carcinoma]]
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = [[salivary gland]], [[breast]], [[lung]], [[skin]], [[trachea]], others
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs = mass
| Symptoms =
| Prevalence = relatively common malignant salivary gland tumour
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis =
| Other =
| ClinDDx =
| Tx = surgical excision
}}
'''Adenoid cystic carcinoma''', abbreviated '''AdCC''', is a malignant tumour commonly seen in the [[salivary gland]].

==General==
*Common malignant neoplasm of salivary gland.<ref name=pmid8949764>{{Cite journal | last1 = Krüll | first1 = A. | last2 = Schwarz | first2 = R. | last3 = Engenhart | first3 = R. | last4 = Huber | first4 = P. | last5 = Lessel | first5 = A. | last6 = Koppe | first6 = H. | last7 = Favre | first7 = A. | last8 = Breteau | first8 = N. | last9 = Auberger | first9 = T. | title = European results in neutron therapy of malignant salivary gland tumors. | journal = Bull Cancer Radiother | volume = 83 Suppl | issue = | pages = 125-9s | month = | year = 1996 | doi = | PMID = 8949764 }}</ref>
*May occur in the skin.<ref name=pmid3010759>{{Cite journal | last1 = Wick | first1 = MR. | last2 = Swanson | first2 = PE. | title = Primary adenoid cystic carcinoma of the skin. A clinical, histological, and immunocytochemical comparison with adenoid cystic carcinoma of salivary glands and adenoid basal cell carcinoma. | journal = Am J Dermatopathol | volume = 8 | issue = 1 | pages = 2-13 | month = Feb | year = 1986 | doi = | PMID = 3010759 }}</ref>
*AKA ''cylindroma''.<ref>Chest. May 1957. Vol. 31. No. 5. PP. 493-511. [http://www.chestjournal.org/content/31/5/493.abstract http://www.chestjournal.org/content/31/5/493.abstract]</ref>
**Should ''not'' be confused with ''[[dermal cylindroma]]'' (a benign skin tumour).
*Composed of ductal cells and myoepithelial cells; '''myoepithelial cells''' > ductal cells.

==Microscopic==
Features:
*[[Cribriform architecture]] ''or'' pseudoglandular spaces (classic pattern) - '''important feature'''.
**Other patterns: solid, cords, (bilayered) tubules.
**Cystic spaces filled with basophilic material (that is PAS +ve) - '''key feature'''.
*Scant cytoplasm in most cells (myoepithelial cells) - clear/eosinophilic.
**Moderate eosinophilic cytoplasm in the (rare) ductal cells.
*Nucleus - small.
**May be angulated (carrot-shaped) - myoepithelial cells; round/ovoid in ductal cells.
*Hyaline stroma.

Memory device:
*A'''d'''CC - mostly '''D'''NA (scant cytoplasm), distinct nucleus (carrot-shaped).

Notes:
*'''Squamous differentiation is extremely rare'''. It presence should prompt consideration of:
**Basaloid [[squamous cell carcinoma]].
**[[Basal cell carcinoma]] (BCC).
*[[Perineural invasion]] is usually present.
**If it is ''not'' one should consider other diagnoses.

DDx:
*[[Pleomorphic adenoma]], esp. if encapsulated.
*[[Epithelial-myoepithelial carcinoma]] - esp. for AdCC tubular variant.
*[[Human papillomavirus-related carcinoma with adenoid cystic-like features]].

===Images===
<gallery>
Image:Adenoid cystic carcinoma - low mag.jpg | AdCC - low mag. (WC/Nephron)
Image:Adenoid_cystic_carcinoma_-_intermed_mag.jpg | AdCC - intermed. mag. (WC/Nephron)
Image:Adenoid_cystic_carcinoma_-_high_mag.jpg | AdCC - high mag. (WC/Nephron)
Image:Adenoid cystic carcinoma - very high mag.jpg | AdCC - very high mag. (WC/Nephron)
Image:Adenoid_cystic_carcinoma_-a-_very_high_mag.jpg | AdCC - very high mag. (WC/Nephron)
File:Breast_AdenoidCysticCarcinoma_SolidType_14BR***.jpg | AdCC of breast - solid type (WC/Sarahkayb)
</gallery>
<gallery>
Image: Adenoid cystic carcinoma of the trachea -- low mag.jpg | AdCC [[trachea]] - low mag.
Image: Adenoid cystic carcinoma of the trachea -- intermed mag.jpg | AdCC trachea - intermed. mag.
Image: Adenoid cystic carcinoma of the trachea -- high mag.jpg | AdCC trachea - high mag.
Image: Adenoid cystic carcinoma of the trachea -- very high mag.jpg | AdCC trachea - very high mag.
Image: Adenoid cystic carcinoma of the trachea - alt -- very high mag.jpg | AdCC trachea - very high mag.
</gallery>
===Grading===
Based on solid component:
*Low grade = tubules and cribriform structures only; no solid component.
*Intermediate grade = solid component <30%.
*High grade = solid component >=30%

==Stains==
Special stains:
*PAS +ve material - cystic spaces.<ref name=pc_add>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5]. Accessed on: 12 May 2011.</ref>

==IHC==
Features:<ref name=pmid19360297>{{Cite journal | last1 = Sequeiros-Santiago | first1 = G. | last2 = García-Carracedo | first2 = D. | last3 = Fresno | first3 = MF. | last4 = Suarez | first4 = C. | last5 = Rodrigo | first5 = JP. | last6 = Gonzalez | first6 = MV. | title = Oncogene amplification pattern in adenoid cystic carcinoma of the salivary glands. | journal = Oncol Rep | volume = 21 | issue = 5 | pages = 1215-22 | month = May | year = 2009 | doi = | PMID = 19360297 }}</ref>
*[[CD117]] +ve.
*Cyclin D1 +ve/-ve.

Others:<ref name=pmid24037641>{{Cite journal | last1 = Thompson | first1 = LD. | last2 = Penner | first2 = C. | last3 = Ho | first3 = NJ. | last4 = Foss | first4 = RD. | last5 = Miettinen | first5 = M. | last6 = Wieneke | first6 = JA. | last7 = Moskaluk | first7 = CA. | last8 = Stelow | first8 = EB. | title = Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases. | journal = Head Neck Pathol | volume = | issue = | pages = | month = Sep | year = 2013 | doi = 10.1007/s12105-013-0487-3 | PMID = 24037641 }}</ref>
*[[CK7]] +ve.
*[[Pankeratin]] +ve.
*[[p63]] +ve.
*Calponin +ve.
*S-100 +ve.

Note:
*Myoepithelial markers (e.g. calponin, actin) +ve.
**Typically -ve in [[PLGA]].

==Molecular==
Features:<ref>{{Cite journal | last1 = Mitani | first1 = Y. | last2 = Rao | first2 = PH. | last3 = Futreal | first3 = PA. | last4 = Roberts | first4 = D. | last5 = Stephens | first5 = P. | last6 = Zhao | first6 = YJ. | last7 = Zhang | first7 = L. | last8 = Mitani | first8 = M. | last9 = Weber | first9 = RS. | title = Novel Chromosomal Rearrangements and breakpoints at the t(6;9) in Salivary Adenoid Cystic Carcinoma: association with MYB-NFIB chimeric fusion, MYB expression, and clinical outcome. | journal = Clin Cancer Res | volume = | issue = | pages = | month = Oct | year = 2011 | doi = 10.1158/1078-0432.CCR-11-1870 | PMID = 21976542 }}</ref>
*t(6;9) MYB-NFIB.
**Seen in ~50% of cases.
**Worse prognosis if present, esp. if fusion assoc. with transcription.

==See also==
*[[Salivary glands]].
*[[Adenoid cystic carcinoma of the breast]].
*[[Adenoid cystic/basal cell carcinoma of the prostate]].
*[[Head and neck pathology]].
*[[Head and neck cytopathology]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Head and neck pathology]]
[[Category:Salivary gland]]

Papillary thyroid carcinoma

2018-04-26T12:57:36Z

Brigitte: /* Microscopic */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Papillary_thyroid_microcarcinoma_-_high_mag.jpg
| Width =
| Caption = Papillary thyroid carcinoma. [[H&E stain]].
| Micro = nuclear changes: nuclear membrane irregularities (e.g. raisinoid shape), +/-nuclear grooves, +/-[[nuclear pseudoinclusions]], +/-nuclear clearing, nuclear enlargement (usu. mild), nucleoli; architectural changes: overlap of nuclei, papillae (not required), +/-[[psammoma bodies]]
| Subtypes = [[Papillary thyroid carcinoma tall cell variant|tall cell variant]], [[Papillary thyroid carcinoma columnar cell variant|columnar cell variant]], [[Papillary thyroid carcinoma follicular variant|follicular variant]], [[Papillary thyroid carcinoma cribriform-morular variant|cribriform-morular variant]], [[Papillary thyroid carcinoma diffuse sclerosing variant|diffuse sclerosing variant]], [[Papillary thyroid carcinoma Warthin-like variant|Warthin-like variant]], [[Papillary thyroid carcinoma solid variant|solid variant]], [[Papillary thyroid carcinoma oncocytic variant|oncocytic variant]], others
| LMDDx = [[lymphocytic thyroiditis]] ([[Graves disease]], [[Hashimoto thyroiditis]]), [[solid cell nest of thyroid]], [[follicular thyroid carcinoma]], [[follicular thyroid adenoma]], [[adenomatoid nodule]], [[noninvasive follicular thyroid neoplasm with papillary-like nuclear features]] (NIFTP)
| Stains =
| IHC = HBME-1 +ve, [[CK19]] +ve, Galectin-3 +ve, thyroglobulin +ve, TTF-1 +ve
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Staging = [[thyroid cancer staging]]
| Site = [[thyroid gland]]
| Assdx =
| Syndromes = [[familial adenomatous polyposis]] (cribriform-morular variant)
| Clinicalhx =
| Signs = thyroid mass
| Symptoms =
| Prevalence = very common
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = usu. good
| Other =
| ClinDDx = other [[thyroid gland]] tumours
}}
'''Papillary thyroid carcinoma''', abbreviated '''PTC''', is the most common [[thyroid gland]] malignancy. It usually has an indolent course.

==General==
Medical school memory device P's:
*Palpable [[lymph nodes]].
*Popular - most common malignant neoplasm of the thyroid.
*Prognosis is good.
*Pre-Tx iodine scan.
*Post-Sx iodine scan.
*[[Psammoma bodies]].

Notes:
*PTC is associated with radiation exposure.<ref name=Ref_Sternberg4_564>{{Ref Sternberg4|564}}</ref>
*''Papillary thyroid microcarcinoma'' is defined as a tumour with a maximal dimension of 1.0 cm or less.<ref name=pmid21267823>{{Cite journal | last1 = Sethom | first1 = A. | last2 = Riahi | first2 = I. | last3 = Riahi | first3 = K. | last4 = Akkari | first4 = K. | last5 = Benzarti | first5 = S. | last6 = Miled | first6 = I. | last7 = Chebbi | first7 = MK. | title = [Management of thyroid microcarcinoma. Report of 13 cases]. | journal = Tunis Med | volume = 89 | issue = 1 | pages = 23-5 | month = Jan | year = 2011 | doi = | PMID = 21267823 }}</ref>

===Prognosis===
Prognosis can be predicted by ''MAICS'' score. It which includes:<ref name=pmid12016468>{{Cite journal | last1 = Hay | first1 = ID. | last2 = Thompson | first2 = GB. | last3 = Grant | first3 = CS. | last4 = Bergstralh | first4 = EJ. | last5 = Dvorak | first5 = CE. | last6 = Gorman | first6 = CA. | last7 = Maurer | first7 = MS. | last8 = McIver | first8 = B. | last9 = Mullan | first9 = BP. | title = Papillary thyroid carcinoma managed at the Mayo Clinic during six decades (1940-1999): temporal trends in initial therapy and long-term outcome in 2444 consecutively treated patients. | journal = World J Surg | volume = 26 | issue = 8 | pages = 879-85 | month = Aug | year = 2002 | doi = 10.1007/s00268-002-6612-1 | PMID = 12016468 }}</ref>
*'''M'''etastases.
*'''A'''ge.
*'''I'''nvasion of surround tissues.
*'''C'''ompleteness of excision.
*'''S'''ize of tumour.

==Microscopic==
Features:
*Nuclear changes - '''key feature'''.
*#"Shrivelled nuclei"/"raisin" like nuclei, nuclei with a wavy ("textured", convoluted) nuclear membrane -- usu. easy to find.
*#[[Nuclear pseudoinclusions]] -- usu. harder to find; have high [[specificity]] (nuclear pseudoinclusions appear as a result of the very convoluted nuclear membrane wrapping around parts of the cytoplasm; true nuclear inclusions in contrast are seen only in viral infections).
*#Nuclear grooves, seen as a result of the highly "textured" nuclear membrane.
*#Nuclear clearing (only on permanent section) - also known as "Orphan Annie eyes".
*Overlap of nuclei - "cells do not respect each other's borders" (easy to see at '''key feature at low power''').
*Classically has papillae (nipple-like shape); papilla (definition): epithelium on fibrovascular core.
**Absence of papillae does not exclude diagnosis.
*[[Psammoma bodies]].
**Circular, acellular, eosinophilic whorled bodies.
**Not necessary to make diagnosis - but very specific in the context of a specimen labeled "thyroid".
**Arise from infarction & calcification of papilla tips.<ref name=Ref_Sternberg4_565>{{Ref Sternberg4|565}}</ref>

Notes:
*Psammoma bodies are awesome if you see 'em, i.e. useful for arriving at the diagnosis.
**If there are no papillae structures -- you're unlikely to see psammoma bodies.
*At low power look for cellular areas/loss of follicles.
*Nuclear clearing seen in:
**Hashimoto's and papillary thyroid carcinoma.<ref name=Ref_Sternberg4_566>{{Ref Sternberg4|566}}</ref>
**May be an artifact of [[fixation]]/processing.
*Nuclear overlapping is easy to see at lower power-- should be the tip-off to look at high power for nuclear features.
*Nuclear inclusions are quite rare and not required to make the diagnosis -- but a very convincing feature if seen.
*Papillae may be seen in Graves disease.
*Thyroid tissue lateral to the jugular vein (often referred to as ''[[lateral aberrant thyroid tissue]]'') is generally considered metastatic thyroid carcinoma (papillary thyroid carcinoma) even if it looks benign.<ref name=pmid14452106>{{Cite journal | last1 = JOHNSON | first1 = RW. | last2 = SAHA | first2 = NC. | title = The so-called lateral aberrant thyroid. | journal = Br Med J | volume = 1 | issue = 5293 | pages = 1668-9 | month = Jun | year = 1962 | doi = | PMID = 14452106 | PMC = 1958877 }}</ref>
**This dictum is disputed.<ref name=pmid17319317>{{Cite journal | last1 = Escofet | first1 = X. | last2 = Khan | first2 = AZ. | last3 = Mazarani | first3 = W. | last4 = Woods | first4 = WG. | title = Lessons to be learned: a case study approach. Lateral aberrant thyroid tissue: is it always malignant? | journal = J R Soc Promot Health | volume = 127 | issue = 1 | pages = 45-6 | month = Jan | year = 2007 | doi = | PMID = 17319317 }}</ref>
**The level VI and VII [[lymph nodes]] are medial to the jugular.

DDx:
*[[Lymphocytic thyroiditis]]:
**[[Graves disease]].
**[[Hashimoto thyroiditis]].
*[[Solid cell nest of thyroid]].<ref name=pmid16830963>{{Cite journal | last1 = Baloch | first1 = ZW. | last2 = LiVolsi | first2 = VA. | title = Cytologic and architectural mimics of papillary thyroid carcinoma. Diagnostic challenges in fine-needle aspiration and surgical pathology specimens. | journal = Am J Clin Pathol | volume = 125 Suppl | issue = | pages = S135-44 | month = Jun | year = 2006 | doi = | PMID = 16830963 | URL = http://ajcp.ascpjournals.org/content/supplements/125/Suppl_1/S135.full.pdf }}</ref>

===Subtypes of papillary thyroid carcinoma===
There are many.

Poor prognosis variants:
*[[Papillary thyroid carcinoma tall cell variant|Tall cell variant]].<ref name=pmid22432054>{{Cite journal | last1 = Gonzalez-Gonzalez | first1 = R. | last2 = Bologna-Molina | first2 = R. | last3 = Carreon-Burciaga | first3 = RG. | last4 = Gómezpalacio-Gastelum | first4 = M. | last5 = Molina-Frechero | first5 = N. | last6 = Salazar-Rodríguez | first6 = S. | title = Papillary thyroid carcinoma: differential diagnosis and prognostic values of its different variants: review of the literature. | journal = ISRN Oncol | volume = 2011 | issue = | pages = 915925 | month = | year = 2011 | doi = 10.5402/2011/915925 | PMID = 22432054 | PMC = 3302055 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/pmid/22432054/?tool=pubmed }}</ref>
*[[Papillary thyroid carcinoma columnar cell variant|Columnar cell variant]].<ref name=pmid22432054/>
*[[Papillary thyroid carcinoma solid variant|Solid variant]].<ref name=pmid22432054/>
*[[Papillary thyroid carcinoma diffuse sclerosing variant|Diffuse sclerosing variant]].<ref>URL: [http://emedicine.medscape.com/article/849000-overview#a0104 http://emedicine.medscape.com/article/849000-overview#a0104]. Accessed on: 1 May 2012.</ref>

====Papillary thyroid carcinoma tall cell variant====
=====General=====
*~10% of PTC.<ref>{{Ref Sternberg5|505}}</ref>
*Often large > 6 cm.

=====Microscopic=====
Features:<ref name=pmid19373912>{{cite journal |author=Urano M, Kiriyama Y, Takakuwa Y, Kuroda M |title=Tall cell variant of papillary thyroid carcinoma: Its characteristic features demonstrated by fine-needle aspiration cytology and immunohistochemical study |journal=Diagn. Cytopathol. |volume= |issue= |pages= |year=2009 |month=April |pmid=19373912 |doi=10.1002/dc.21086 |url=}}</ref>
*50% of cells with height 2x the width.<ref name=pmid18925842>{{cite journal |author=Ghossein R, Livolsi VA |title=Papillary thyroid carcinoma tall cell variant |journal=Thyroid |volume=18 |issue=11 |pages=1179–81 |year=2008 |month=November |pmid=18925842 |doi=10.1089/thy.2008.0164 |url=}}</ref>
**There is some disagreement on these criteria;<ref name=pmid18925842/> Raphael believes the height ought to be ~3x width, for 50% of the cells.<ref>S. Raphael. 17 January 2011.</ref>
*Eosinophilic cytoplasm.
*Well-defined cell borders.
*Nucleus stratified; basal location, i.e. closer to the basement membrane.

Negative:
*Nuclei ''not'' pseudostratified, if pseudostratified consider ''columnar cell variant''.

Images:
<gallery>
Image:Papillary_thyroid_carcinoma_tall_cell_var_intermed_mag.jpg | PTC tall cell variant - intermed. mag. (WC)
Image:Papillary_thyroid_carcinoma_tall_cell_var_high_mag.jpg | PTC tall cell variant - high mag. (WC)
</gallery>

====Papillary thyroid carcinoma columnar cell variant====
=====General=====
Epidemiology:
*Poor prognosis.
*Very rare.

=====Microscopic=====
Features:<ref name=Ref_Sternberg5_506>{{Ref Sternberg5|506}}</ref>
*Elongated nuclei (similar to colorectal adenocarcinoma) - '''key feature'''.
*+/-Pseudostratification of the nuclei (like in colorectal adenocarcinoma), differentiates from ''tall cell variant''.
*Nuclear stratification - '''key feature'''.
*"Minimal" papillary features.
*"Tall cells".
*Clear-eosinophilic cytoplasm.
*Mitoses common.

Image: [http://www3.interscience.wiley.com/cgi-bin/fulltext/75000320/nfig003a?CRETRY=1&SRETRY=0 Columnar variant PTC (wiley.com)].
====Papillary thyroid carcinoma follicular variant====
=====General=====
*May be confused with [[follicular thyroid carcinoma|follicular carcinoma]] or [[follicular thyroid adenoma|follicular adenoma]].
*Pathologists often disagree about this diagnosis.<ref name=pmid21940284>{{Cite journal | last1 = Daniels | first1 = GH. | title = What if many follicular variant papillary thyroid carcinomas are not malignant? A review of follicular variant papillary thyroid carcinoma and a proposal for a new classification. | journal = Endocr Pract | volume = 17 | issue = 5 | pages = 768-87 | month = | year = | doi = 10.4158/EP10407.RA | PMID = 21940284 }}</ref>

=====Microscopic=====
Features:<ref name=Ref_EP88>{{Ref EP|88}}</ref>
*Small tightly packed follicles - '''key feature'''.
*Hypereosinophilic colloid.
*Nuclear features of PTC.
**Large nuclei.
**Typically have less [[nuclear pseudoinclusion]]s than the conventional type.
*+/-Fibrous capsule (common).

DDx:
*[[Noninvasive follicular thyroid neoplasm with papillary-like nuclear features]] (NIFTP).
*[[Follicular thyroid carcinoma]] - has a fibrous capsule and invasion though it.
*[[Follicular thyroid adenoma]] - surrounded by a fibrous capsule.
*[[Adenomatoid nodule]] - round nuclei, no nuclear features of PTC.

Images:

===Papillary thyroid carcinoma cribriform-morular variant===
=====General=====
*Associated with [[familial adenomatous polyposis]] (FAP).<ref name=pmid18612695>{{cite journal |author=Groen EJ, Roos A, Muntinghe FL, ''et al.'' |title=Extra-intestinal manifestations of familial adenomatous polyposis |journal=Ann. Surg. Oncol. |volume=15 |issue=9 |pages=2439–50 |year=2008 |month=September |pmid=18612695 |pmc=2518080 |doi=10.1245/s10434-008-9981-3 |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2518080/?tool=pubmed}}</ref>

=====Microscopic=====
Features:
*Circumscribed or even encapsulated neoplasm.
*Morules - interspersed balls of squamoid cells
**No keritinization or intercellular bridges.
**Homogenous, lightly eosinophilic glassy nuclei (biotin accumulation).
*Follicles
**[[Cribriform]], papillary, trabecular and solid patterns.
**Columnar or cuboidal cells.
**Little colloid
**Papillary carcinoma nuclear features.

<gallery>
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant MP3 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - Medium power (SKB)
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant HP2 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - High power (SKB)
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant HP3 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - high power]]
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant P16 HP3 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - High power (SKB)
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant CDX2 HP 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - CDX2 (SKB)
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant P16 HP 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - p16 (SKB)
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant ER HP 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - ER (SKB)
Image:Thyroid PapillaryCarcinoma CribriformMorularVariant betaCatenin HP 13BR***.jpg|Thyroid - Papillary Carcinoma Cribriform Morular Variant - beta catenin (SKB)
</gallery>

DDX:
*Papillary thyroid carcinoma
*Papillary thyroid carcinoma, tall cell variant

=====IHC=====
*CDX2 - Highlights the morules (CDX2 is positive in the biotin rich nuclei associated with morule formation in a variety of situations)<ref>{{Cite journal | last1 = Wani | first1 = Y. | last2 = Notohara | first2 = K. | last3 = Nakatani | first3 = Y. | last4 = Matsuzaki | first4 = A. | title = Aberrant nuclear Cdx2 expression in morule-forming tumours in different organs, accompanied by cytoplasmic reactivity. | journal = Histopathology | volume = 55 | issue = 4 | pages = 465-8 | month = Oct | year = 2009 | doi = 10.1111/j.1365-2559.2009.03382.x | PMID = 19817898 }}</ref>
*CD10 - Highlights the morules <ref>{{Cite journal | last1 = Cameselle-Teijeiro | first1 = J. | last2 = Alberte-Lista | first2 = L. | last3 = Chiarelli | first3 = S. | last4 = Buriticá | first4 = C. | last5 = Gonçalves | first5 = L. | last6 = González-Cámpora | first6 = R. | last7 = Nogales | first7 = FF. | title = CD10 is a characteristic marker of tumours forming morules with biotin-rich, optically clear nuclei that occur in different organs. | journal = Histopathology | volume = 52 | issue = 3 | pages = 389-92 | month = Feb | year = 2008 | doi = 10.1111/j.1365-2559.2007.02911.x | PMID = 18081818 }}</ref>
*Beta-catenin - nuclear and cytoplasmic - all tumour cells.
*Estrogen receptor - positive
*TTF-1 - positive

=====Molecular=====
*Up-regulating disturbances in the Wnt signaling pathway promote formation of morules with optically clear biotin rich nuclei <ref>{{Cite journal | last1 = Gamachi | first1 = A. | last2 = Kashima | first2 = K. | last3 = Daa | first3 = T. | last4 = Nakatani | first4 = Y. | last5 = Tsujimoto | first5 = M. | last6 = Yokoyama | first6 = S. | title = Aberrant intranuclear localization of biotin, biotin-binding enzymes, and beta-catenin in pregnancy-related endometrium and morule-associated neoplastic lesions. | journal = Mod Pathol | volume = 16 | issue = 11 | pages = 1124-31 | month = Nov | year = 2003 | doi = 10.1097/01.MP.0000092953.20717.48 | PMID = 14614052 }}</ref>
**Mutation of the beta-catenin gene
**Mutation in APC
*Examples
***Well-differentiated fetal adenocarcinoma
***Papillary thyroid carcinoma, cribriform morular variant (mutation in APC in familial variants)
***Pancreatoblastoma

====Papillary thyroid carcinoma diffuse sclerosing variant====
=====General=====
*Usually young adults, children.

=====Microscopic=====
Features:<ref>{{Ref PBoD8|1122}}</ref>
*Papillae - usu. prominent.
*Squamous morules - '''key features'''.<ref name=pmid15233643>{{Cite journal | last1 = Hirokawa | first1 = M. | last2 = Kuma | first2 = S. | last3 = Miyauchi | first3 = A. | last4 = Qian | first4 = ZR. | last5 = Nakasono | first5 = M. | last6 = Sano | first6 = T. | last7 = Kakudo | first7 = K. | title = Morules in cribriform-morular variant of papillary thyroid carcinoma: Immunohistochemical characteristics and distinction from squamous metaplasia. | journal = APMIS | volume = 112 | issue = 4-5 | pages = 275-82 | month = | year = | doi = 10.1111/j.1600-0463.2004.apm11204-0508.x | PMID = 15233643 }}
</ref>
*Lymphocytes - abundant.
*Fibrosis.

DDx:
*Lymphocytic thyroiditis (esp. Hashimoto's thyroiditis).

====Papillary thyroid carcinoma Warthin-like variant====
*Resembles [[Warthin tumour]].
=====Microscopic=====
Features:<ref name=Ref_Sternberg5_506>{{Ref Sternberg5|506}}</ref>
*Eosinophilic cytoplasm.
*Lymphocytic thyroiditis.
*Papillae.

====Papillary thyroid carcinoma solid variant====
Features:<ref name=pmid22432054/>
*Some studies suggest this has a poor prognosis.
*More common in children.
*Associated with Chernobyl nuclear accident.

=====Microscopic=====
Features:
*Solid sheets >50% of tumour mass.<ref name=pmid22432054/>

====Papillary thyroid carcinoma oncocytic variant====
Features:
*Possible association with [[autoimmune thyroiditis]].<ref name=pmid9013831>{{Cite journal | last1 = Berho | first1 = M. | last2 = Suster | first2 = S. | title = The oncocytic variant of papillary carcinoma of the thyroid: a clinicopathologic study of 15 cases. | journal = Hum Pathol | volume = 28 | issue = 1 | pages = 47-53 | month = Jan | year = 1997 | doi = | PMID = 9013831 }}</ref>

=====Microscopic=====
Features:<ref name=pmid9013831/>
*Abundant oncocytic tumour cells with apical nuclei.
*Classic features of PTC:
**Grooves and and abundant pseudoinclusions.<ref name=Ref_EP86>{{Ref EP|86}}</ref>
*>70% papillary architecture.<ref name=Ref_EP86>{{Ref EP|86}}</ref>
*+/-Degenerative changes.

Note:
*CK19 +ve -- though ''not'' specific or sensitive.

<gallery>
Image: Papillary thyroid carcinoma oncocytic variant -- low mag.jpg | PTC oncocytic - low mag. (WC)
Image: Papillary thyroid carcinoma oncocytic variant -- intermed mag.jpg | PTC oncocytic - intermed mag. (WC)
Image: Papillary thyroid carcinoma oncocytic variant -- high mag.jpg | PTC oncocytic - high mag. (WC)
Image: Papillary thyroid carcinoma oncocytic variant -- very high mag.jpg | PTC oncocytic - very high mag. (WC)
</gallery>

===IHC===
Thyroid versus something else:
*Thyroglobulin +ve.<ref name=pmid23637102>{{Cite journal | last1 = Sathiyamoorthy | first1 = S. | last2 = Maleki | first2 = Z. | title = Cytomorphologic overlap of differentiated thyroid carcinoma and lung adenocarcinoma and diagnostic value of TTF-1 and TGB on cytologic material. | journal = Diagn Cytopathol | volume = 42 | issue = 1 | pages = 5-10 | month = Jan | year = 2014 | doi = 10.1002/dc.22997 | PMID = 23637102 }}</ref>
*TTF-1 ([[thyroid transcription factor-1]]) +ve.
*CD15 +ve.{{fact}}

PTC versus benign:<ref>{{Cite journal | last1 = Mataraci | first1 = EA. | last2 = Ozgüven | first2 = BY. | last3 = Kabukçuoglu | first3 = F. | title = Expression of cytokeratin 19, HBME-1 and galectin-3 in neoplastic and nonneoplastic thyroid lesions. | journal = Pol J Pathol | volume = 63 | issue = 1 | pages = 58-64 | month = Mar | year = 2012 | doi = | PMID = 22535608 }}</ref>
*HBME-1 +ve (strong, diffuse).
*[[CK19]] +ve (strong, diffuse).
*Galectin-3 +ve (strong, diffuse).

===Molecular===
*Currently not widely used in a diagnostic context.

====Tabular summary====
Molecular changes in papillary thyroid carcinoma as per ''Adeniran et al'':<ref name=pmid16434896>{{Cite journal | last1 = Adeniran | first1 = AJ. | last2 = Zhu | first2 = Z. | last3 = Gandhi | first3 = M. | last4 = Steward | first4 = DL. | last5 = Fidler | first5 = JP. | last6 = Giordano | first6 = TJ. | last7 = Biddinger | first7 = PW. | last8 = Nikiforov | first8 = YE. | title = Correlation between genetic alterations and microscopic features, clinical manifestations, and prognostic characteristics of thyroid papillary carcinomas. | journal = Am J Surg Pathol | volume = 30 | issue = 2 | pages = 216-22 | month = Feb | year = 2006 | doi = | PMID = 16434896 }}</ref>
{| class="wikitable sortable"
! Molecular change
! Frequency
! Histology
! Notes
|-
|BRAF point mutations
| ~ 40%
| [[papillary thyroid carcinoma tall cell variant|tall cell variant]]
| poorer prognosis, older individuals
|-
|RET/PTC rearrangments
| ~ 20%
| papillary architecture, [[psammoma bodies]]
| younger individuals
|-
|RAS point mutations
| ~ 15%
| exclusively [[papillary thyroid carcinoma follicular variant|follicular variant]]
| -
|}

==Sign out==
<pre>
HEMITHYROID, RIGHT, COMPLETION OF TOTAL THYROIDECTOMY:
- PAPILLARY THYROID CARCINOMA, FOLLICULAR VARIANT.
-- TUMOUR SIZE: 4 MM (MAXIMAL).
-- ARCHITECTURE: FOLLICULAR.
-- CYTOMORPHOLOGY: CLASSICAL.
-- HISTOLOGIC GRADE: G1 (WELL DIFFERENTIATED).
-- NO TUMOUR CAPSULE IDENTIFIED.
-- NEGATIVE FOR LYMPHOVASCULAR INVASION.
-- NEGATIVE FOR PERINEURAL INVASION.
-- NEGATIVE FOR EXTRATHYROIDAL EXTENSION.
-- SURGICAL MARGINS NEGATIVE FOR MALIGNANCY.
</pre>

Note:
*If it is a completion thyroidectomy and the staging changes one should do a full synoptic report.

===Microcarcinoma===
<pre>
A. LEFT HEMITHYROID, THYROIDECTOMY COMPLETION:
- PAPILLARY THYROID MICROCARCINOMA.
-- MARGINS NEGATIVE FOR MALIGNANCY.
-- TUMOUR SIZE ~ 1 MILLIMETRE.
-- NEGATIVE FOR LYMPHOVASCULAR INVASION.
-- NEGATIVE FOR PERINEURAL INVASION.
- PALPATION THYROIDITIS, FOCAL.
- NODULAR HYPERPLASIA.

B. LYMPH NODES, LEVEL 6 AND 7, LYMPH NODE DISSECTION:
- TWO LYMPH NODES, NEGATIVE FOR MALIGNANCY ( 0 POSITIVE / 2 ).
</pre>

<pre>
THYROID GLAND, TOTAL THYROIDECTOMY:
- INCIDENTAL PAPILLARY THYROID MICROCARCINOMA.
-- MARGINS NEGATIVE FOR MALIGNANCY.
-- TUMOUR SIZE ~ 1 MILLIMETRE.
-- NEGATIVE FOR LYMPHOVASCULAR INVASION.
-- NEGATIVE FOR PERINEURAL INVASION.
- NODULAR HYPERPLASIA.
- ONE PARATHYROID GLAND.
</pre>

===Lymph node dissection===
<pre>
A. NECK, RIGHT LEVEL 2 AND 3, LYMPH NODE DISSECTION:
- ONE LYMPH NODE POSITIVE FOR PAPILLARY THYROID CARCINOMA ( 1 POSITIVE / 4 ).

B. NECK, RIGHT LEVEL 4, LYMPH NODE DISSECTION:
- TWO LYMPH NODES, NEGATIVE FOR MALIGNANCY ( 0 POSITIVE / 2 ).

C. NECK, RIGHT LEVEL 6 AND 7, LYMPH NODE DISSECTION:
- ONE LYMPH NODE POSITIVE FOR PAPILLARY THYROID CARCINOMA ( 1 POSITIVE / 3 ).
</pre>

====Micro====
The sections show lymph nodes with tumour that has a papillary architecture. The
tumour cell nuclei are enlarged and overlap. They also have nuclear grooves, nucleoli
and abundant pseudoinclusions. The chromatin of the tumour cells has a powdery
appearance.

==See also==
*[[Thyroid gland]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Thyroid gland]]

Medullary thyroid carcinoma

2018-04-26T12:49:25Z

Brigitte: /* Images */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Medullary thyroid carcinoma - 2 - high mag.jpg
| Width =
| Caption = Medullary thyroid carcinoma. [[H&E stain]].
| Synonyms =
| Micro = nuclei with neuroendocrine features (round nuclei with salt-and-pepper chromatin), +/-[[amyloid]] deposits (fluffy appearing acellular eosinophilic material), +/-[[C-cell hyperplasia]]
| Subtypes =
| LMDDx =
| Stains = congo red +ve (amyloid deposits)
| IHC = calcitonin +ve, [[CEA]] +ve, chromogranin A +ve, synaptophysin +ve, thyroglobulin -ve (usually)
| EM =
| Molecular =
| IF =
| Gross = usu. well-circumscribed, white, gray or yellow, gritty, firm
| Grossing =
| Staging = [[thyroid cancer staging]]
| Site = [[thyroid gland]]
| Assdx = [[C-cell hyperplasia]]
| Syndromes = [[multiple endocrine neoplasia IIa]], [[multiple endocrine neoplasia IIb]]
| Clinicalhx =
| Signs =
| Symptoms =
| Prevalence = uncommon
| Bloodwork = +/-serum calcitonin elevated
| Rads =
| Endoscopy =
| Prognosis = poor
| Other =
| ClinDDx =
| Tx =
}}
'''Medullary thyroid carcinoma''', abbreviated '''MTC''', is an uncommon epithelial [[malignancy]] of the thyroid gland that may be syndromic.

==General==
Medical school memory device - 3 M's:
*[[amyloid|aMyloid]].
*Median node dissection done.
*[[MEN IIa syndrome]]/[[MEN IIb syndrome]].
**Medullary thyroid carcinoma.
**[[Pheochromocytoma]].
**[[Parathyroid adenoma]].

Epidemiology:
*Very rare.
*Poor prognosis.
*May be genetic (MEN IIa/b syndrome).
*Arises from C cells (which produce calcitonin).

Sporadic tumours
*~80%
*Slightly older age at presentation (~45)
*Tend to be solitary

Syndromic tumours - typically:<ref name=pmid21455198>{{Cite journal | last1 = Nosé | first1 = V. | title = Familial thyroid cancer: a review. | journal = Mod Pathol | volume = 24 Suppl 2 | issue = | pages = S19-33 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.147 | PMID = 21455198 |URL = http://www.nature.com/modpathol/journal/v24/n2s/full/modpathol2010147a.html }}</ref>
*Present in 30s or 40s.
*+/-Multifocal.
*+/-Bilateral.
*[[C-cell hyperplasia]].

Serology:
*Serum calcitonin classically elevated.<ref name=pmid24470736>{{Cite journal | last1 = Vainas | first1 = I. | last2 = Marthopoulos | first2 = A. | last3 = Chrisoulidou | first3 = A. | last4 = Raptou | first4 = K. | last5 = Tziomalos | first5 = K. | last6 = Pazaitou-Panayiotou | first6 = K. | title = Calcitonin stimulation tests for the early diagnosis and follow-up of patients with C cell disease: a descriptive analysis. | journal = Hippokratia | volume = 17 | issue = 3 | pages = 246-51 | month = Jul | year = 2013 | doi = | PMID = 24470736 }}</ref>
*CEA may also be elevated.

==Gross==
Features:<ref name=pmid21455198/>
*Usu. well-circumscribed.
*White, gray or yellow.
*Gritty.
*Firm.

Image:
*[http://www.nature.com/modpathol/journal/v24/n2s/fig_tab/modpathol2010147f2.html MTC (nature.com)].

==Microscopic==
Architecture - various
*Nested with delicate vascular septa
*Trabecular
*Tubular/glandular
*Pseudo-papillary

Cells
*Polygonal to spindle to small cells
*Amphophilic, somewhat granular cytoplasm
*Cells may have a more bizarre appearance
*Cells may appear to be 'falling apart'due to interstitial oedema.

Stroma
*+/-[[Amyloid]] deposits - fluffy appearing acellular eosinophilic material in the cytoplasm.
*Stroma is vascular and can show haemorrhage, hyalinised collagen, oedema or metaplastic bone
*Coarse calcification
*True psammoma bodies may be present

Nuclei
*Nuclei with "neuroendocrine features".
**Small, round nuclei.
**Coarse chromatin (''salt and pepper nuclei'').

Surrounding Thyroid
*+/-[[C-cell hyperplasia]] - seen with familial forms of MTC.
**C cells (AKA ''parafollicular cell''): abundant cytoplasm - clear/pale.

Note:
*The amyloid is formed from ''calcitonin''.<ref name=pmid15459123>{{Cite journal | last1 = Khurana | first1 = R. | last2 = Agarwal | first2 = A. | last3 = Bajpai | first3 = VK. | last4 = Verma | first4 = N. | last5 = Sharma | first5 = AK. | last6 = Gupta | first6 = RP. | last7 = Madhusudan | first7 = KP. | title = Unraveling the amyloid associated with human medullary thyroid carcinoma. | journal = Endocrinology | volume = 145 | issue = 12 | pages = 5465-70 | month = Dec | year = 2004 | doi = 10.1210/en.2004-0780 | PMID = 15459123 }}</ref>

DDx:
*Other thyroid tumours:
**[[Anaplastic thyroid carcinoma]].
**[[Papillary thyroid carcinoma]].
**[[Hurthle cell carcinoma]]
***The oncocytic variant of medullary carcinoma can be confused with [[Hurthle cell carcinoma]]. Clues to suggest medullary carcinoma:
****Cytoplasm is amphophilic as opposed to eosinophilic
****Nests of tumour cells separated by fibrous septa
*[[C-cell hyperplasia]].
**Invasive medullary carcinoma shows fibrosis around tumor cells and stains more weakly for calcitonin.
*Other neuroendocrine tumours - primary or metastatic:
**[[Paraganglioma]] - negative for keratin, calcitonin and [[CEA]].
**[[Carcinoid]] - negative for calcitonin.
*Metastatic [[melanoma]].
**Pigment.
**Melanoma markers positive, calcitonin and CEA negative.

===Images===
www:
*[http://www.anatomyatlases.org/MicroscopicAnatomy/Images/Plate287.jpg Parafollicular cells (anatomyatlases.org)].
<gallery>
Image:Medullary_thyroid_carcinoma_-_low_mag.jpg | MTC - low mag. (WC)
Image:Medullary_thyroid_carcinoma_-_high_mag.jpg | MTC - high mag. (WC)
Image:Medullary_thyroid_carcinoma_-_2_-_high_mag.jpg | MTC and amyloid - high mag. (WC)
Image:Thyroid MedullaryCarcinoma 216 PA.JPG|Thyroid - Medullary carcinoma - low power (SKB)
Image:Thyroid MedullaryCarcinoma 217 PA.JPG|Thyroid - Medullary carcinoma - low power (SKB)
Image:Thyroid MedullaryCarcinoma Amyloid RBWH.JPG|Thyroid - Medullary carcinoma - amyloid (SKB)
Image:Thyroid MedullaryCarcinoma Amyloid MP3 PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB)
Image:Thyroid MedullaryCarcinoma Amyloid MP2 PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB)
Image:Thyroid MedullaryCarcinoma Amyloid MP4 PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB)
Image:Thyroid MedullaryCarcinoma Amyloid MP PA.JPG|Thyroid - Medullary carcinoma - amyloid - medium power (SKB)
Image:Thyroid MedullaryCarcinoma Amyloid HP PA.JPG|Thyroid - Medullary carcinoma - amyloid - high power (SKB)
Image:Thyroid MedullaryCarcinoma Comedonecrosis LP2 CTR.jpg|Thyroid - Medullary carcinoma - comedonecrosis - low power (SKB)
Image:Thyroid MedullaryCarcinoma Comedonecrosis LP CTR.jpg|Thyroid - Medullary carcinoma - comedonecrosis - medium power (SKB)
Image:Thyroid MedullaryCarcinoma Comedonecrosis HP CTR.jpg|Thyroid - Medullary carcinoma - comedonecrosis - high power (SKB)
Image:Thyroid MedullaryCarcinoma SpindleCell LP PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - low power (SKB)
Image:Thyroid MedullaryCarcinoma SpindleCell MP PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - medium power (SKB)
Image:Thyroid MedullaryCarcinoma SpindleCell LP2 PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - low power (SKB)
Image:Thyroid MedullaryCarcinoma SpindleCell HP PA.JPG|Thyroid - Medullary carcinoma - Spindle cell variant - high power (SKB)
Image:Thyroid MedullaryCarcinoma SmallCell HP PA.JPG|Thyroid - Medullary carcinoma - Small cell variant - high power (SKB)
Image:Thyroid MedullaryCarcinoma SmallCellVariant MP CTR.jpg|Thyroid - Medullary carcinoma - small cell variant - medium power (SKB)
Image:Thyroid MedullaryCarcinoma SmallCellVariant HP CTR (3).jpg|Thyroid - Medullary carcinoma - small cell variant - high power (SKB)
Image:Thyroid MedullaryCarcinoma MP CTR (2).jpg|Thyroid - Medullary carcinoma - medium power (SKB)
Image:Thyroid MedullaryCarcinoma HP2 CTR.jpg|Thyroid - Medullary carcinoma - high power (SKB)
</gallery>

==Stains==
*Congo-red +ve (amyloid present) - mnemonic: ''CRAP'' -- congo red amyloid protein.

==IHC==
Features:<ref>URL: [http://pathologyoutlines.com/thyroid.html#medullary http://pathologyoutlines.com/thyroid.html#medullary]. Accessed on: 17 January 2011.</ref>
*[[Calcitonin]] +ve - it arises from C cells (which produce calcitonin).
*Neuroendocrine markers.
**[[Chromogranin A]].
**[[Synaptophysin]].
*[[CEA]] +ve (often better staining than calcitonin).<ref>SB. 7 January 2010.</ref>
*Thyroglobulin usu. -ve.<ref name=pmid8454270>{{Cite journal | last1 = de Micco | first1 = C. | last2 = Chapel | first2 = F. | last3 = Dor | first3 = AM. | last4 = Garcia | first4 = S. | last5 = Ruf | first5 = J. | last6 = Carayon | first6 = P. | last7 = Henry | first7 = JF. | last8 = Lebreuil | first8 = G. | title = Thyroglobulin in medullary thyroid carcinoma: immunohistochemical study with polyclonal and monoclonal antibodies. | journal = Hum Pathol | volume = 24 | issue = 3 | pages = 256-62 | month = Mar | year = 1993 | doi = | PMID = 8454270 }}</ref>
*TTF-1 +ve

==EM==
*Neurosecretory granules.
**Feature seen in neuroendocrine tumours.

Images: [http://pathhsw5m54.ucsf.edu/case7/image77.html Neurosecretory granules (ucsf.edu)].

==Sign out==
<pre>
Lesion, Liver, Core Biopsy:
- METASTATIC MEDULLARY THYROID CARCINOMA, see comment.

Comment:
Stains/IHC confirm the morphologic findings; the tumour stains as follows:
POSITIVE: calcitonin, CEAp, synaptophysin, CD56, TTF-1 (focal, moderate), congo red (confirms the presence of amyloid).
NEGATIVE: thyroglobulin, CDX2.
</pre>

===Micro===
The sections show cells of intermediate size without apparent nucleoli, moderate eosinophilic cytoplasm, arranged in nests, focally associated with amorphous acellular cotton candy-like material.

The cotton candy-like material has a light apple-green appearance when polarized.

==See also==
*[[Thyroid gland]].
*[[Medullary carcinoma]].

==References==
{{reflist|2}}

[[Category:Thyroid gland]]
[[Category:Diagnosis]]

Mucoepidermoid carcinoma

2018-04-26T02:25:59Z

Brigitte: /* Images */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Mucoepidermoid_carcinoma_-_2a_-_very_high_mag.jpg
| Width =
| Caption = Mucoepidermoid carcinoma. [[H&E stain]].
| Micro = mucous cells (abundant fluffy cytoplasm and large mucin vacuoles - nucleus distorted by mucin vacuole, cells may be scarce); epidermoid cells (non-keratinized, polygonal squamoid cell with clear or oncocytic cytoplasm); architecture - cystic (low grade) or solid (high grade)
| Subtypes =
| LMDDx = [[squamous cell carcinoma of the head and neck]], [[adenosquamous carcinoma]], [[pleomorphic adenoma]]
| Stains = mucous cells: [[alcian blue stain]] +ve, [[mucicarmine stain]] +ve
| IHC =
| EM =
| Molecular = t(11;19)(q21;p13)
| IF =
| Gross = solid, cystic or both
| Grossing =
| Site = [[salivary gland]], classically parotid gland
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs = mass lesion
| Symptoms =
| Prevalence = most common malignant salivary gland tumour, generally uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis =
| Other =
| ClinDDx =
}}
'''Mucoepidermoid carcinoma''', abbreviated '''MEC''', the is the most common malignant neoplasm of the [[salivary gland]].

==General==
*Most common malignant neoplasm of salivary gland in all age groups.<ref>URL: [http://path.upmc.edu/cases/case715/dx.html http://path.upmc.edu/cases/case715/dx.html]. Accessed on: 2 February 2012.</ref><ref name=pmid16003616>{{Cite journal | last1 = Bell | first1 = RB. | last2 = Dierks | first2 = EJ. | last3 = Homer | first3 = L. | last4 = Potter | first4 = BE. | title = Management and outcome of patients with malignant salivary gland tumors. | journal = J Oral Maxillofac Surg | volume = 63 | issue = 7 | pages = 917-28 | month = Jul | year = 2005 | doi = | PMID = 16003616 }}</ref>
*Female:male ~= 3:2.
*Site: parotid > submandibular.

==Gross==
*Cystic or solid, usu. a mix of both.

==Microscopic==
Features:
*Architecture:<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/D2A001-PQ01-M.htm http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/D2A001-PQ01-M.htm]. Accessed on: 19 October 2010.</ref>
**Cystic (low grade).
**Solid (high grade).
*Mucous cells with abundant fluffy cytoplasm and large mucin vacuoles - '''key feature'''.
**Nucleus distorted by mucin vacuole.
**Mucous cell may be scarce - more difficult to diagnose.
*Epidermoid cells:
**Non-keratinized, polygonal squamoid cell with clear or oncocytic cytoplasm.
***Clear cells contain glycogen ([[PAS]] +ve, [[PAS-D]] -ve).

Notes:
*The classic description - composed of 3 cell types: epidermoid, intermediate, and mucin producing.<ref>{{Cite journal | last1 = Lennerz | first1 = JK. | last2 = Perry | first2 = A. | last3 = Mills | first3 = JC. | last4 = Huettner | first4 = PC. | last5 = Pfeifer | first5 = JD. | title = Mucoepidermoid carcinoma of the cervix: another tumor with the t(11;19)-associated CRTC1-MAML2 gene fusion. | journal = Am J Surg Pathol | volume = 33 | issue = 6 | pages = 835-43 | month = Jun | year = 2009 | doi = 10.1097/PAS.0b013e318190cf5b | PMID = 19092631 }}</ref>
**"Intermediate cells" are described in textbooks. Weinreb thinks they are a pretty much a myth.<ref name=IW_10jan2011>IW. 10 January 2011.</ref>
*Mucin vacuoles may be rare; in a superficial glance -- it may mimic [[squamous cell carcinoma]].
*The thought of high-grade MEC should prompt consideration of squamous cell carcinoma.

DDx:<ref name=pmid22262946>{{Cite journal | last1 = Mokhtari | first1 = S. | last2 = Mokhtari | first2 = S. | title = Clinical features and differential diagnoses in laryngeal mucoepidermoid carcinoma. | journal = Clin Med Insights Pathol | volume = 5 | issue = | pages = 1-6 | month = | year = 2012 | doi = 10.4137/CPath.S8435 | PMID = 22262946 }}</ref>
*[[Squamous cell carcinoma of the skin]] - 75% of metastases to the parotid region are from the skin.<ref name=pmid2694753>{{Cite journal | last1 = Zanetti | first1 = D. | last2 = Renaldini | first2 = G. | last3 = Peretti | first3 = G. | last4 = Antonelli | first4 = AR. | title = [Intra-parotid lymph node metastasis of malignant skin neoplasms of the head]. | journal = Acta Otorhinolaryngol Ital | volume = 9 | issue = 4 | pages = 381-90 | month = | year = | doi = | PMID = 2694753 }}</ref>
*[[Squamous cell carcinoma of the head and neck]].
*[[Adenosquamous carcinoma]].
*[[Pleomorphic adenoma]].<ref name=pmid15754364>{{Cite journal | last1 = Siddiqui | first1 = NH. | last2 = Wu | first2 = SJ. | title = Fine-needle aspiration biopsy of cystic pleomorphic adenoma with adnexa-like differentiation mimicking mucoepidermoid carcinoma: a case report. | journal = Diagn Cytopathol | volume = 32 | issue = 4 | pages = 229-32 | month = Apr | year = 2005 | doi = 10.1002/dc.20215 | PMID = 15754364 }}</ref>

===Images===
<gallery>
Image:Mucoepidermoid_carcinoma_%282%29_HE_stain.jpg | Mucoepidermoid carcinoma 2. (WC)
Image:Mucoepidermoid_carcinoma_%283%29_HE_stain.jpg | Mucoepidermoid carcinoma 3. (WC)
Image:Mucoepidermoid_carcinoma_-_2_-_intermed_mag.jpg | Mucoepidermoid carcinoma - 2 - intermed. mag. (WC/Nephron)
Image:Mucoepidermoid_carcinoma_-_2_-_high_mag.jpg | Mucoepidermoid carcinoma - 2 - high mag. (WC/Nephron)
Image:Mucoepidermoid_carcinoma_-_2a_-_very_high_mag.jpg | Mucoepidermoid carcinoma - 2 - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3256999/figure/f2-cpath-5-2012-001/ Low-grade MEC (nih.gov)].<ref name=pmid22262946>{{Cite journal | last1 = Mokhtari | first1 = S. | last2 = Mokhtari | first2 = S. | title = Clinical features and differential diagnoses in laryngeal mucoepidermoid carcinoma. | journal = Clin Med Insights Pathol | volume = 5 | issue = | pages = 1-6 | month = | year = 2012 | doi = 10.4137/CPath.S8435 | PMID = 22262946 }}</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3256999/figure/f3-cpath-5-2012-001/ Intermed. grade MEC (nih.gov)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3256999/figure/f4-cpath-5-2012-001/ High-grade MEC (nih.gov)].

[[File: 5_20985699291686_sl 1.png|Mucoepidermoid carcinoma of parotid, low grade]]
[[File: 5_20985699291686_sl 2.png|Mucoepidermoid carcinoma of parotid, low grade]]
[[File: 5_20985699291686_sl 3.png|Mucoepidermoid carcinoma of parotid, low grade]]
[[File: 5_20985699291686_sl 4.png|Mucoepidermoid carcinoma of parotid, low grade]]

Low grade mucoepidermoid carcinoma in left parotid of 51 year old woman. A. Tumor shows blue squamoud differentiation with cystic dilatation. B This focus shows more obvious cyst formation. C. Squamous component with round to spindled cancer cells; note that currently squamous pearls and high grade squamous cancer elements render the tumor a squamous carcinoma, according to Barnes. D. Glandular cell component.

===Subtypes===
*Conventional.
*Oncocytic.
**Definition: composed of 50% oncocytes.
**Good outcome.<ref name=pmid18971778>{{cite journal |author=Weinreb I, Seethala RR, Perez-Ordoñez B, Chetty R, Hoschar AP, Hunt JL |title=Oncocytic mucoepidermoid carcinoma: clinicopathologic description in a series of 12 cases |journal=Am. J. Surg. Pathol. |volume=33 |issue=3 |pages=409–16 |year=2009 |month=March |pmid=18971778 |doi=10.1097/PAS.0b013e318184b36d |url=}}</ref>
*Clear cell.
*Unicystic (cystadenocarcinoma).
**Based on the gross. (???)
*Sclerosing MEC +/- eosinophilia.
**Rare.

===Grading===
General:
*Two competing system exist:
**AFIP.<ref name=pmid9529011>{{cite journal |author=Goode RK, Auclair PL, Ellis GL |title=Mucoepidermoid carcinoma of the major salivary glands: clinical and histopathologic analysis of 234 cases with evaluation of grading criteria |journal=Cancer |volume=82 |issue=7 |pages=1217–24 |year=1998 |month=April |pmid=9529011 |doi= |url=}}</ref>
**Brandwein.<ref name=pmid11420454>{{cite journal |author=Brandwein MS, Ivanov K, Wallace DI, ''et al.'' |title=Mucoepidermoid carcinoma: a clinicopathologic study of 80 patients with special reference to histological grading |journal=Am. J. Surg. Pathol. |volume=25 |issue=7 |pages=835–45 |year=2001 |month=July |pmid=11420454 |doi= |url=}}</ref>

Notes:
*Both systems have their pros and cons.
*Weinreb uses the AFIP system with a slight modification.

====AFIP====
#Low cystic component (<20%) - 2 points.
#[[Perineural invasion]] - 2 points.
#[[Necrosis]] - 3 points.
#Mitoses > 4 per 10 HPFs (HPF not defined in paper - see [[HPFitis]]) - 3 points.
#Anaplasia - 4 points.

Scoring:
*Low grade = 0-4 points.
*Intermediate grade = 5-6 points.
*High grade = 7+ points.

=====Weinreb modification=====
Weinreb looks for the following:
*Tumour invades in small nests/islands - 2 points.
**If applicable, the two points are added to the AFIP score.
**The tumour is graded using the AFIP (scoring) cut points -- see above.

Notes:
*It seems pointless to memorize this but it is occasionally asked on exams.
**How to remember: think of the Nottingham grading system (architecture, mitoses, nuclear grade) + necrosis + LVI.

==Stains==
Mucous cells:
*Alcian blue +ve.
*Mucicarmine +ve.

Notes:
*Above useful for MEC versus [[squamous cell carcinoma]].<ref name=pmid23960993>{{Cite journal | last1 = Jastrzebski | first1 = A. | last2 = Brownstein | first2 = S. | last3 = Jordan | first3 = DR. | last4 = Gilberg | first4 = SM. | title = Histochemical analysis and immunohistochemical profile of mucoepidermoid carcinoma of the conjunctiva. | journal = Saudi J Ophthalmol | volume = 26 | issue = 2 | pages = 205-10 | month = Apr | year = 2012 | doi = 10.1016/j.sjopt.2012.01.004 | PMID = 23960993 }}</ref>

==IHC==
*CK7 +ve.
*CK20 -ve.
*CEA +ve.<ref>{{Cite journal | last1 = Hassanin | first1 = MB. | last2 = Ghosh | first2 = L. | last3 = Das | first3 = AK. | last4 = Waterhouse | first4 = JP. | title = Immunohistochemical and fluorescent microscopic study of histogenesis of salivary mucoepidermoid carcinoma. | journal = J Oral Pathol Med | volume = 18 | issue = 5 | pages = 291-8 | month = May | year = 1989 | doi = | PMID = 2475619 }}</ref>

==Molecular==
*t(11;19)(q21;p13) -- MECT1-MAML2 fusion.<ref name=pmid12539049>{{cite journal |author=Tonon G, Modi S, Wu L, ''et al.'' |title=t(11;19)(q21;p13) translocation in mucoepidermoid carcinoma creates a novel fusion product that disrupts a Notch signaling pathway |journal=Nat. Genet. |volume=33 |issue=2 |pages=208–13 |year=2003 |month=February |pmid=12539049 |doi=10.1038/ng1083 |url=}}</ref><ref name=pmid20588178>{{cite journal |author=Seethala RR, Dacic S, Cieply K, Kelly LM, Nikiforova MN |title=A reappraisal of the MECT1/MAML2 translocation in salivary mucoepidermoid carcinomas |journal=Am. J. Surg. Pathol. |volume=34 |issue=8 |pages=1106–21 |year=2010 |month=August |pmid=20588178 |doi=10.1097/PAS.0b013e3181de3021 |url=}}</ref>
**Present in ~65% of MECs.
**Presence assoc. with low-grade MEC (vs. high-grade MEC) & favourable prognosis.
**Not seen in tumours that are in the DDx of MEC.

==See also==
*[[Salivary glands]].
*[[Squamous cell carcinoma]].
*[[Sclerosing mucoepidermoid carcinoma with eosinophilia]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Salivary gland]]

Acinic cell carcinoma

2018-04-26T02:06:16Z

Brigitte: /* www */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Acinic cell carcinoma -- very high mag.jpg
| Width =
| Caption = Acinic cell carcinoma. [[H&E stain]].
| Micro = "acinic cells" (abundant finely vacuolated cytoplasm with basophilic granules, small nuclei with stippled chromatin), scattered "intercalcated duct type cells" (eosinophilic cytoplasm with moderate amount of cytoplasm and bland nuclei), +/-peri-tumoural lymphocytes, +/-glassy extracellular bluish/purple blobs
| Subtypes = oncocytic variant, clear cell variant, papillary cystic variant
| LMDDx = [[oncocytoma of the salivary gland]], [[mucoepidermoid carcinoma]], adenocarcinoma NOS, [[mammary analogue secretory carcinoma]]
| Stains = PAS +ve, PASD +ve
| IHC = p63 -ve, S-100 -ve
| EM = [[zymogen granules]]
| Molecular =
| IF =
| Gross = tan or reddish
| Grossing =
| Site = [[salivary gland]] - usu. parotid gland
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs = salivary gland mass
| Symptoms =
| Prevalence = uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = good
| Other =
| ClinDDx =
}}
'''Acinic cell carcinoma''', abbreviated '''AcCC''', is a rare type of [[salivary gland]] cancer. It is also known as '''acinic cell adenocarcinoma'''.

It should '''not''' to be confused with ''[[pancreatic acinar cell carcinoma]]''.

==General==
*Malignant neoplasm of salivary gland arising from acinic cells.
*The relative prevalence of the neoplasm in the various salivary gland reflects the abundance of acinic cells: parotid gland (~80%) > minor salivary glands (~17%) > submandibular glands (~3%).
*Affects wide age range -- including children.
*Site affect prognosis (most aggressive to least aggressive): submandibular > parotid > minor salivary.
*Good prognosis - in one large cohort:<ref name=pmid23754708>{{Cite journal | last1 = Patel | first1 = NR. | last2 = Sanghvi | first2 = S. | last3 = Khan | first3 = MN. | last4 = Husain | first4 = Q. | last5 = Baredes | first5 = S. | last6 = Eloy | first6 = JA. | title = Demographic trends and disease-specific survival in salivary acinic cell carcinoma: an analysis of 1129 cases. | journal = Laryngoscope | volume = 124 | issue = 1 | pages = 172-8 | month = Jan | year = 2014 | doi = 10.1002/lary.24231 | PMID = 23754708 }}</ref>
**~97% five year survival.
**~93% 10 year survival.

==Gross==
*Tan or reddish.

==Microscopic==
Features:
*Sheets of acinic cells with:
**Abundant finely vacuolated cytoplasm with basophilic granules - '''key feature'''.
***Granules may be focal.
**Small nuclei stippled chromatin.
*Scattered intercalcated duct type cells with:
**Eosinophilic cytoplasm with moderate amount of cytoplasm.
**Bland nuclei with slightly larger than seen in acinic cells.
*+/-Peri-tumoural lymphocytes.
*+/-Glassy extracellular bluish/purple blobs.

Notes:
*Adipose tissue -- present in the salivary glands -- is absent in AcCC.
*May focally resemble thyroid tissue.
*Smaller (characteristic) microvacuoles (unreported in the literature) may be present that have a bubbly appearance and glassy basophilic inclusions.<ref name=IW_10jan2011>IW. 11 January 2011.</ref>

Memory device:
*AcCC - lots of "C"s - '''c'''hromatin stipled, '''c'''ytoplasm generous.

DDx:
*[[Oncocytoma of the salivary gland]].
*Adenocarcinoma not otherwise specified.<ref name=pmid12608654>{{Cite journal | last1 = Ihrler | first1 = S. | last2 = Blasenbreu-Vogt | first2 = S. | last3 = Sendelhofert | first3 = A. | last4 = Lang | first4 = S. | last5 = Zietz | first5 = C. | last6 = Löhrs | first6 = U. | title = Differential diagnosis of salivary acinic cell carcinoma and adenocarcinoma (NOS). A comparison of (immuno-)histochemical markers. | journal = Pathol Res Pract | volume = 198 | issue = 12 | pages = 777-83 | month = | year = 2002 | doi = | PMID = 12608654 }}</ref>
*[[Mammary analogue secretory carcinoma]].<ref name=pmid22127547>{{Cite journal | last1 = Lei | first1 = Y. | last2 = Chiosea | first2 = SI. | title = Re-evaluating historic cohort of salivary acinic cell carcinoma with new diagnostic tools. | journal = Head Neck Pathol | volume = 6 | issue = 2 | pages = 166-70 | month = Jun | year = 2012 | doi = 10.1007/s12105-011-0312-9 | PMID = 22127547 }}</ref>

===Images===
====Case====
<gallery>
Image:Acinic_cell_carcinoma_-_intermed_mag.jpg | AcCC - intermed. mag. (WC/Nephron)
Image:Acinic_cell_carcinoma_-_high_mag.jpg | AcCC - high mag. (WC/Nephron)
Image:Acinic_cell_carcinoma_-_very_high_mag.jpg | AcCC - very high mag. (WC/Nephron)
</gallery>

====Case====
<gallery>
Image: Acinic cell carcinoma -- low mag.jpg |AcCC - low mag. (WC/Nephron)
Image: Acinic cell carcinoma -- intermed mag.jpg |AcCC - intermed. mag. (WC/Nephron)
Image: Acinic cell carcinoma -- high mag.jpg |AcCC - high mag. (WC/Nephron)
Image: Acinic cell carcinoma -- very high mag.jpg |AcCC - very high mag. (WC/Nephron)
Image: Acinic cell carcinoma - alt -- very high mag.jpg |AcCC - very high mag. (WC/Nephron)
</gallery>

====www====
*[http://www.brown.edu/Courses/Digital_Path/systemic_path/hn/acinic.html AcCC (brown.edu)].

===Grading===
General:
*Not prognostic.
*Done to avoid phone calls from clinician.

Factors Weinreb uses:<ref name=IW_10jan2011>IW. 11 January 2011.</ref>
*[[Necrosis]].
*Nuclear atypia.
*[[Perineural invasion]].
*[[Mitoses]].
*Infiltrative margin.
*Tumour sclerosis.

===Subtypes===
*Oncocytic variant - rare.
*Clear cell variant - rare.
*Papillary cystic variant.

==Stains==
*PAS +ve.
*PAS-D +ve.

==IHC==
*S-100 -ve.
*p63 -ve (30 -ve of 30 cases<ref name=pmid23054955/>).
**p63 +ve in [[mucoepidermoid carcinoma]] (24 +ve of 24 cases).<ref name=pmid23054955>{{Cite journal | last1 = Sams | first1 = RN. | last2 = Gnepp | first2 = DR. | title = P63 expression can be used in differential diagnosis of salivary gland acinic cell and mucoepidermoid carcinomas. | journal = Head Neck Pathol | volume = 7 | issue = 1 | pages = 64-8 | month = Mar | year = 2013 | doi = 10.1007/s12105-012-0403-2 | PMID = 23054955 }}</ref>

There are a bunch of other [[stains]] that are touted to be useful (amylase, anti-chymotrypsin, lactoferrin). Weinreb thinks these are '''not''' helpful.<ref name=IW_10jan2011>IW. 11 January 2011.</ref>

==EM==
*[[Zymogen granules]].<ref name=pmid14991547>{{Cite journal | last1 = Sun | first1 = Y. | last2 = Wasserman | first2 = PG. | title = Acinar cell carcinoma arising in the stomach: a case report with literature review. | journal = Hum Pathol | volume = 35 | issue = 2 | pages = 263-5 | month = Feb | year = 2004 | doi = | PMID = 14991547 }}</ref>

==Sign out==
<pre>
A. Tumour, Left Parotid Gland, Excision:
- ACINIC CELL CARCINOMA, see comment.
-- Margins clear.
-- Please see synoptic report.

B. Lymph Nodes, Left - Level 2 & 3, Lymphadenectomy:
- Five lymph nodes NEGATIVE for malignancy (0/5).

Comment:
Immunostains are in keeping with acinic cell carcinoma (mammoglobin, S-100 and p63 are all NEGATIVE). A PAS stain is positive in the tumour.
</pre>

==See also==
*[[Salivary glands]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Salivary gland]]
[[Category:Head and neck pathology]]

Acinic cell carcinoma

2018-04-26T02:05:33Z

Brigitte: /* www */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Acinic cell carcinoma -- very high mag.jpg
| Width =
| Caption = Acinic cell carcinoma. [[H&E stain]].
| Micro = "acinic cells" (abundant finely vacuolated cytoplasm with basophilic granules, small nuclei with stippled chromatin), scattered "intercalcated duct type cells" (eosinophilic cytoplasm with moderate amount of cytoplasm and bland nuclei), +/-peri-tumoural lymphocytes, +/-glassy extracellular bluish/purple blobs
| Subtypes = oncocytic variant, clear cell variant, papillary cystic variant
| LMDDx = [[oncocytoma of the salivary gland]], [[mucoepidermoid carcinoma]], adenocarcinoma NOS, [[mammary analogue secretory carcinoma]]
| Stains = PAS +ve, PASD +ve
| IHC = p63 -ve, S-100 -ve
| EM = [[zymogen granules]]
| Molecular =
| IF =
| Gross = tan or reddish
| Grossing =
| Site = [[salivary gland]] - usu. parotid gland
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs = salivary gland mass
| Symptoms =
| Prevalence = uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = good
| Other =
| ClinDDx =
}}
'''Acinic cell carcinoma''', abbreviated '''AcCC''', is a rare type of [[salivary gland]] cancer. It is also known as '''acinic cell adenocarcinoma'''.

It should '''not''' to be confused with ''[[pancreatic acinar cell carcinoma]]''.

==General==
*Malignant neoplasm of salivary gland arising from acinic cells.
*The relative prevalence of the neoplasm in the various salivary gland reflects the abundance of acinic cells: parotid gland (~80%) > minor salivary glands (~17%) > submandibular glands (~3%).
*Affects wide age range -- including children.
*Site affect prognosis (most aggressive to least aggressive): submandibular > parotid > minor salivary.
*Good prognosis - in one large cohort:<ref name=pmid23754708>{{Cite journal | last1 = Patel | first1 = NR. | last2 = Sanghvi | first2 = S. | last3 = Khan | first3 = MN. | last4 = Husain | first4 = Q. | last5 = Baredes | first5 = S. | last6 = Eloy | first6 = JA. | title = Demographic trends and disease-specific survival in salivary acinic cell carcinoma: an analysis of 1129 cases. | journal = Laryngoscope | volume = 124 | issue = 1 | pages = 172-8 | month = Jan | year = 2014 | doi = 10.1002/lary.24231 | PMID = 23754708 }}</ref>
**~97% five year survival.
**~93% 10 year survival.

==Gross==
*Tan or reddish.

==Microscopic==
Features:
*Sheets of acinic cells with:
**Abundant finely vacuolated cytoplasm with basophilic granules - '''key feature'''.
***Granules may be focal.
**Small nuclei stippled chromatin.
*Scattered intercalcated duct type cells with:
**Eosinophilic cytoplasm with moderate amount of cytoplasm.
**Bland nuclei with slightly larger than seen in acinic cells.
*+/-Peri-tumoural lymphocytes.
*+/-Glassy extracellular bluish/purple blobs.

Notes:
*Adipose tissue -- present in the salivary glands -- is absent in AcCC.
*May focally resemble thyroid tissue.
*Smaller (characteristic) microvacuoles (unreported in the literature) may be present that have a bubbly appearance and glassy basophilic inclusions.<ref name=IW_10jan2011>IW. 11 January 2011.</ref>

Memory device:
*AcCC - lots of "C"s - '''c'''hromatin stipled, '''c'''ytoplasm generous.

DDx:
*[[Oncocytoma of the salivary gland]].
*Adenocarcinoma not otherwise specified.<ref name=pmid12608654>{{Cite journal | last1 = Ihrler | first1 = S. | last2 = Blasenbreu-Vogt | first2 = S. | last3 = Sendelhofert | first3 = A. | last4 = Lang | first4 = S. | last5 = Zietz | first5 = C. | last6 = Löhrs | first6 = U. | title = Differential diagnosis of salivary acinic cell carcinoma and adenocarcinoma (NOS). A comparison of (immuno-)histochemical markers. | journal = Pathol Res Pract | volume = 198 | issue = 12 | pages = 777-83 | month = | year = 2002 | doi = | PMID = 12608654 }}</ref>
*[[Mammary analogue secretory carcinoma]].<ref name=pmid22127547>{{Cite journal | last1 = Lei | first1 = Y. | last2 = Chiosea | first2 = SI. | title = Re-evaluating historic cohort of salivary acinic cell carcinoma with new diagnostic tools. | journal = Head Neck Pathol | volume = 6 | issue = 2 | pages = 166-70 | month = Jun | year = 2012 | doi = 10.1007/s12105-011-0312-9 | PMID = 22127547 }}</ref>

===Images===
====Case====
<gallery>
Image:Acinic_cell_carcinoma_-_intermed_mag.jpg | AcCC - intermed. mag. (WC/Nephron)
Image:Acinic_cell_carcinoma_-_high_mag.jpg | AcCC - high mag. (WC/Nephron)
Image:Acinic_cell_carcinoma_-_very_high_mag.jpg | AcCC - very high mag. (WC/Nephron)
</gallery>

====Case====
<gallery>
Image: Acinic cell carcinoma -- low mag.jpg |AcCC - low mag. (WC/Nephron)
Image: Acinic cell carcinoma -- intermed mag.jpg |AcCC - intermed. mag. (WC/Nephron)
Image: Acinic cell carcinoma -- high mag.jpg |AcCC - high mag. (WC/Nephron)
Image: Acinic cell carcinoma -- very high mag.jpg |AcCC - very high mag. (WC/Nephron)
Image: Acinic cell carcinoma - alt -- very high mag.jpg |AcCC - very high mag. (WC/Nephron)
</gallery>

====www====
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802170246445 AcCC (surgicalpathologyatlas.com)].
*[http://www.brown.edu/Courses/Digital_Path/systemic_path/hn/acinic.html AcCC (brown.edu)].

===Grading===
General:
*Not prognostic.
*Done to avoid phone calls from clinician.

Factors Weinreb uses:<ref name=IW_10jan2011>IW. 11 January 2011.</ref>
*[[Necrosis]].
*Nuclear atypia.
*[[Perineural invasion]].
*[[Mitoses]].
*Infiltrative margin.
*Tumour sclerosis.

===Subtypes===
*Oncocytic variant - rare.
*Clear cell variant - rare.
*Papillary cystic variant.

==Stains==
*PAS +ve.
*PAS-D +ve.

==IHC==
*S-100 -ve.
*p63 -ve (30 -ve of 30 cases<ref name=pmid23054955/>).
**p63 +ve in [[mucoepidermoid carcinoma]] (24 +ve of 24 cases).<ref name=pmid23054955>{{Cite journal | last1 = Sams | first1 = RN. | last2 = Gnepp | first2 = DR. | title = P63 expression can be used in differential diagnosis of salivary gland acinic cell and mucoepidermoid carcinomas. | journal = Head Neck Pathol | volume = 7 | issue = 1 | pages = 64-8 | month = Mar | year = 2013 | doi = 10.1007/s12105-012-0403-2 | PMID = 23054955 }}</ref>

There are a bunch of other [[stains]] that are touted to be useful (amylase, anti-chymotrypsin, lactoferrin). Weinreb thinks these are '''not''' helpful.<ref name=IW_10jan2011>IW. 11 January 2011.</ref>

==EM==
*[[Zymogen granules]].<ref name=pmid14991547>{{Cite journal | last1 = Sun | first1 = Y. | last2 = Wasserman | first2 = PG. | title = Acinar cell carcinoma arising in the stomach: a case report with literature review. | journal = Hum Pathol | volume = 35 | issue = 2 | pages = 263-5 | month = Feb | year = 2004 | doi = | PMID = 14991547 }}</ref>

==Sign out==
<pre>
A. Tumour, Left Parotid Gland, Excision:
- ACINIC CELL CARCINOMA, see comment.
-- Margins clear.
-- Please see synoptic report.

B. Lymph Nodes, Left - Level 2 & 3, Lymphadenectomy:
- Five lymph nodes NEGATIVE for malignancy (0/5).

Comment:
Immunostains are in keeping with acinic cell carcinoma (mammoglobin, S-100 and p63 are all NEGATIVE). A PAS stain is positive in the tumour.
</pre>

==See also==
*[[Salivary glands]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Salivary gland]]
[[Category:Head and neck pathology]]

Pediatric gastrointestinal pathology

2018-04-12T12:30:15Z

Brigitte: /* Microscopic */

This article deals with '''pediatric gastrointestinal pathology'''. An introduction to pediatric pathology is in the ''[[pediatric pathology]]'' article.

An overview of (adult) gastrointestinal pathology is in the ''[[gastrointestinal pathology]]'' article.

=Birth defects=
==Omphalocele==
===General===
Usually genetic (unlike [[gastroschisis]]) - associated with:<ref name=pmid20809116>{{Cite journal | last1 = Frolov | first1 = P. | last2 = Alali | first2 = J. | last3 = Klein | first3 = MD. | title = Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature. | journal = Pediatr Surg Int | volume = 26 | issue = 12 | pages = 1135-48 | month = Dec | year = 2010 | doi = 10.1007/s00383-010-2701-7 | PMID = 20809116 }}</ref>
*[[Trisomy 18]] (Edwards syndrome) ~ 6% have omphaloceles in a series of 85 cases.<ref name=pmid3191615>{{Cite journal | last1 = Moore | first1 = CA. | last2 = Harmon | first2 = JP. | last3 = Padilla | first3 = LM. | last4 = Castro | first4 = VB. | last5 = Weaver | first5 = DD. | title = Neural tube defects and omphalocele in trisomy 18. | journal = Clin Genet | volume = 34 | issue = 2 | pages = 98-103 | month = Aug | year = 1988 | doi = | PMID = 3191615 }}</ref>
*[[Beckwith-Wiedemann syndrome]].

Presentation:
*Increased AFP.

===Gross===
*Bowel outside of abdomen - covered by membrane/in a sac.

Image:
*[http://library.med.utah.edu/WebPath/PEDHTML/PED006.html Omphalocele (utah.edu)].

==Gastroschisis==
===General===
*Defect considered to be more severe than [[omphalocele]].
*Usually sporadic.

===Gross===
*Bowel outside of abdomen - individual loops of bowel are seen.

Image:
*[http://med.brown.edu/pedisurg/Brown/IBImages/AbdWallDefects/Gastroschisis%202.html Gastroschisis (brown.edu)].

=Luminal pathology=
==Esophageal atresia==
===General===
*Multifactoral.
*Often associated with other abnormalities.

Forms:<ref name=pmid17498283>{{Cite journal | last1 = Spitz | first1 = L. | title = Oesophageal atresia. | journal = Orphanet J Rare Dis | volume = 2 | issue = | pages = 24 | month = | year = 2007 | doi = 10.1186/1750-1172-2-24 | PMID = 17498283 }}</ref>
#Esophageal atresia with distal tracheoesophageal fistula - most common.
#Esophageal atresia without a fistula.

Note:
*The "H-type" tracheoeosphageal fistula is often lumped together with ''esophageal atresia''.<ref name=pmid17498283/>

Image:
*[http://commons.wikimedia.org/wiki/File:Atrezja.jpg Esophageal atresia (WC)].

==Abetalipoproteinemia==
*[[AKA]] ''Bassen-Kornzweig syndrome''.

===General===
*Rare genetic disorder.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/200100 http://www.ncbi.nlm.nih.gov/omim/200100]. Accessed on: 6 April 2011.</ref><ref>{{cite journal |author=Bassen FA, Kornzweig AL |title=Malformation of the erythrocytes in a case of atypical retinitis pigmentosa |journal=Blood |volume=5 |issue=4 |pages=381–87 |year=1950 |month=April |pmid=15411425 |doi= |url=}}</ref>
*GI-related symptoms similar to [[celiac disease]] - malabsorption.

Clinical features:<ref name=pmid24139731>{{Cite journal | last1 = Hammer | first1 = MB. | last2 = El Euch-Fayache | first2 = G. | last3 = Nehdi | first3 = H. | last4 = Feki | first4 = M. | last5 = Maamouri-Hicheri | first5 = W. | last6 = Hentati | first6 = F. | last7 = Amouri | first7 = R. | title = Clinical features and molecular genetics of two Tunisian families with abetalipoproteinemia. | journal = J Clin Neurosci | volume = 21 | issue = 2 | pages = 311-5 | month = Feb | year = 2014 | doi = 10.1016/j.jocn.2013.04.016 | PMID = 24139731 }}</ref>
*Failure to thrive.
*Pigmented retinopathy.

Blood work:<ref name=pmid24139731/>
*Cholesterol - low.
*Triglyceride - low.
*Apolipoprotein B - very low.

===Microscopic===
Features:
*Enterocytes have clear cytoplasm (due to lipid accumulation).

Notes:
*Have abnormal erythrocytes with a spiculated cell membranes ''acanthocyte'' - seen on blood films.

====Images====
<gallery>
Image:Abetalipoproteinemia_-_intermed_mag.jpg | Abetalipoproteinemia - intermed. mag. (WC)
Image:Abetalipoproteinemia_-_very_high_mag.jpg | Abetalipoproteinemia - very high mag. (WC)
</gallery>

==Microvillous inclusion disease==
*[[AKA]] ''Davidson disease''.
===General===
*Autosomal recessive inherited condition - due to mutation in ''MYO5B''.<ref name=pmid18724368>{{cite journal |author=Müller T, Hess MW, Schiefermeier N, ''et al.'' |title=MYO5B mutations cause microvillus inclusion disease and disrupt epithelial cell polarity |journal=Nat. Genet. |volume=40 |issue=10 |pages=1163–5 |year=2008 |month=October |pmid=18724368 |doi=10.1038/ng.225 |url=}}</ref>

===Microscopic===
Features:
*Flat mucosa; no villi.

Notes:
*Appearance similar to [[celiac sprue]]; however, usually lacks the intraepithelial lymphocytic infiltration characteristic of [[celiac sprue]].

Images:
*[http://path.upmc.edu/cases/case163.html Microvillous inclusion disease - several images (upmc.edu)].

===IHC===
*[[Carcinoembryonic antigen]] (CEA) +ve.<ref name=Ref_Sternberg4>{{Ref Sternberg4|}}</ref>

===EM===
*Diagnosis is dependent on [[electron microscopy]].<ref name=pmid11251929>{{cite journal |author=Kennea N, Norbury R, Anderson G, Tekay A |title=Congenital microvillous inclusion disease presenting as antenatal bowel obstruction |journal=Ultrasound Obstet Gynecol |volume=17 |issue=2 |pages=172–4 |year=2001 |pmid=11251929 |doi=10.1046/j.1469-0705.2001.00211.x}}</ref>

Images:
*[http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=1398 MID (gfmer.ch)].

==Cystic fibrosis==
{{Main|Cystic fibrosis}}

===General===
*Genetic.
*May lead to [[meconium ileus]].

===Microscopic===
Features - large bowel:<ref name=pmid710839>{{cite journal |author=Neutra MR, Trier JS |title=Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture |journal=Gastroenterology |volume=75 |issue=4 |pages=701–10 |year=1978 |month=October |pmid=710839 |doi= |url=}}</ref>
*Crypt enlargement.

Notes:
*''Not'' intracellular and extracellular accumulation of mucus. (?)

==Colonic aganglionosis==
*[[AKA]] ''Hirschsprung disease''.
===General===
*Genetic disorder:<ref>{{OMIM|142623}}</ref>
**5-10% familial; RET gene most commonly mutated.
**Several genes involved.
**Inheritance pattern variable.
*Treatment: surgery (''Swenson's procedure'' or ''Duhamel procedure'').<ref name=pmid6649901>{{Cite journal | last1 = Okasora | first1 = T. | last2 = Okamoto | first2 = E. | last3 = Kuwata | first3 = K. | last4 = Toyosaka | first4 = A. | last5 = Ohashi | first5 = S. | last6 = Ueki | first6 = S. | title = Serum and erythrocyte acetylcholine esterase in Hirschsprung's disease. | journal = Z Kinderchir | volume = 38 | issue = 5 | pages = 298-300 | month = Oct | year = 1983 | doi = 10.1055/s-2008-1059992 | PMID = 6649901 }}</ref>

Pathology:
*Failure of neural crest cell migration
**Parasympathetic ganglion cells in submucosal plexus (Meissner plexus) and myenteric plexus (Auerbach plexus) - absent.<ref name=pmid17139897>{{Cite journal | last1 = Vorobyov | first1 = GI. | last2 = Achkasov | first2 = SI. | last3 = Biryukov | first3 = OM. | title = Hirschsprung's disease in adults. | journal = Acta Chir Iugosl | volume = 53 | issue = 2 | pages = 113-6 | month = | year = 2006 | doi = | PMID = 17139897 }}</ref>

Notes:
*Most common reason for litigation in paediatric pathology.<ref>Taylor, G. 19 January 2011.</ref>

===Gross===
Features:
*Dilated bowel; stuffed sausage-look.

Classification:
*Short-segment (75-80%): Rectum, distal sigmoid
*Long-segment HD (10-20%): Beyond splenic flexure
*Total colonic aganglionosis (5-15%): Entire colon.<ref name=pmid25395999>{{Cite web | last = | first = | title = Diagnosis of Hirschsprung's disease with particular emphasis on histopathology. A systematic review of current literature | url = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/ | publisher = | date = | accessdate = 19 August 2017 }}</ref>

**[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/figure/F0001/ Classification of Hirschsprung's disease according to the aganglionic segment length (https://www.ncbi.nlm.nih.gov)]

Image:
*[http://www.pathology.pitt.edu/lectures/gi/colon-a/03.htm Hirschsprung disease (pathology.pitt.edu)].

===Microscopic===
Features:
*Ganglion cells missing in submucosal plexus and myenteric plexus.
**Increasing ganglia proximally into transition zone
*Hypertrophy of neural plexuses.
**Many nerve trunks > 40 μm
*Abnormal submucosal blood vessels may be seen
*+/-Submucosal fibrosis.

Image:
*[http://pathology.mc.duke.edu/research/Histo_course/myent_plexus.jpg Normal myenteric plexus (duke.edu)].<ref>URL: [http://pathology.mc.duke.edu/research/PTH225.html http://pathology.mc.duke.edu/research/PTH225.html]. Accessed on: 11 January 2011.</ref>

===Stains===
*Acetylcholinesterase - marks the abundant, disorganized, nerve fibers.

Image:
*[http://commons.wikimedia.org/wiki/File:Hirschsprung_acetylcholine.jpg Hirschsprung - acetylcholinesterase (WC)].

===IHC===
Features:<ref name=pmid1640323>{{Cite journal | last1 = Luider | first1 = TM. | last2 = van Dommelen | first2 = MW. | last3 = Tibboel | first3 = D. | last4 = Meijers | first4 = JH. | last5 = Ten Kate | first5 = FJ. | last6 = Trojanowski | first6 = JQ. | last7 = Molenaar | first7 = JC. | title = Differences in phosphorylation state of neurofilament proteins in ganglionic and aganglionic bowel segments of children with Hirschsprung's disease. | journal = J Pediatr Surg | volume = 27 | issue = 7 | pages = 815-9 | month = Jul | year = 1992 | doi = | PMID = 1640323 }}</ref>
*NF-M (neurofilament middle) - highlight hypertrophic nerve fascicules
*NF-H (neurofilament high) - highlight hypertrophic nerve fascicules.
*Tau ~ highlights ganglion cells (which are absent in segments affected by Hirschsprung).<ref name=pmid8229560>{{Cite journal | last1 = Deguchi | first1 = E. | last2 = Iwai | first2 = N. | last3 = Goto | first3 = Y. | last4 = Yanagihara | first4 = J. | last5 = Fushiki | first5 = S. | title = An immunohistochemical study of neurofilament and microtubule-associated Tau protein in the enteric innervation in Hirschsprung's disease. | journal = J Pediatr Surg | volume = 28 | issue = 7 | pages = 886-90 | month = Jul | year = 1993 | doi = | PMID = 8229560 }}</ref>
**Nerve fibres +ve control.

Others<ref>{{Cite book | last1 = Lin | first1 = Fan | last2 = Prichard | first2 = Jeffrey | title = Handbook of practical immunohistochemistry : frequently asked question | date = | publisher = | location = | isbn = 9781493915774 | pages = }}</ref>
:
*Calretinin ~ -ve, Usually negative in hypertrophied nerve fibers in Hirschsprung’s disease, superior to acetylcholinesterase.
*NSE ~ -ve, Highlights ganglion cells to exclude Hirschsprung’s disease; specific but not very sensitive.

==Meconium ileus==
===General===
*Classically due to ''[[cystic fibrosis]]''.
*May lead to ''[[meconium peritonitis]]''.
*Can be mimicked by [[CMV]] infection.<ref>{{cite journal |author=Déchelotte PJ, Mulliez NM, Bouvier RJ, Vanlieféringhen PC, Lémery DJ |title=Pseudo-meconium ileus due to cytomegalovirus infection: a report of three cases |journal=Pediatr Pathol |volume=12 |issue=1 |pages=73–82 |year=1992 |pmid=1313975 |doi= |url=}}</ref>

===Gross===
Features:
*Thick.
**High viscosity.
*Green.

====Image====
*[http://library.med.utah.edu/WebPath/jpeg3/PERI175.jpg Meconium ileus (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html]. Accessed on: 3 December 2011.</ref>

===Microscopic===
Features:
*Meconium-laden macrophages. (???)

==Meconium peritonitis==
===General===
*May be due to a number of causes:
**Aganglionosis ([[Hirschsprung disease]]).
**[[Meconium ileus]].

===Microscopic===
Features:
*Brown granular material - '''key feature'''.
*+/-Multinucleated giant cells.
*Inflammatory infiltrate (PMNs, lymphocytes, plasma cells).

Image:
*[http://www.pathologyoutlines.com/caseofweek/case2008106image2.jpg Meconium peritonitis - gross (pathologyoutlines.com)].

==Necrotizing enterocolitis==
*Abbreviated ''NEC''.
===General===
*Disease primarily of premature babies.
*Diagnosed by imaging.

Note:
*''Enterocolitis'' = inflammation of [[small bowel]] and [[colon]].<ref>URL: [http://medical-dictionary.thefreedictionary.com/enterocolitis http://medical-dictionary.thefreedictionary.com/enterocolitis]. Accessed on: 10 October 2011.</ref>
**''Necrotizing enteritis'' = small bowel only.

===Microscopic===
Features:
*Large spaces.

DDx:
*[[Pneumatosis intestinalis]].

Images:
*[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)].
*[https://library.med.utah.edu/WebPath/PEDHTML/PED045.html NEC - micro].

==Autoimmune enteropathy==
{{Main|Autoimmune enteropathy}}

=Pancreas=
{{Main|Pancreas}}
==Pancreatic islet cell hyperplasia==
===General===
*Associated with maternal [[diabetes]].

===Microscopic===
Features:
*Marked size variation of pancreatic islets.
**Normal islets ~ 150 micrometers (diameter). Hyperplastic islets - up to ~500 micrometers (diameter).

Image:
*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].

=Liver=
{{Main|Liver pathology}}
==Giant cell hepatitis==
*[[AKA]] ''neonatal giant cell hepatitis'', abbreviated ''NGCH''.
===General===
*Rare.

Etiology:
*Unknown - possibly viral, autoimmune and/or drugs.<ref name=pmid21043007>{{Cite journal | last1 = Hartl | first1 = J. | last2 = Buettner | first2 = R. | last3 = Rockmann | first3 = F. | last4 = Farkas | first4 = S. | last5 = Holstege | first5 = A. | last6 = Vogel | first6 = C. | last7 = Schnitzbauer | first7 = A. | last8 = Schlitt | first8 = HJ. | last9 = Schoelmerich | first9 = J. | title = Giant cell hepatitis: an unusual cause of fulminant liver failure. | journal = Z Gastroenterol | volume = 48 | issue = 11 | pages = 1293-6 | month = Nov | year = 2010 | doi = 10.1055/s-0029-1245476 | PMID = 21043007 }}</ref>
*One large series suggests that, in the neonatal population, with follow-up the causes are:
**~49% idiopathic.
**~16% pan-hypopituitarism.
**~8% biliary atresia.
**~6% Alagille syndrome
**~6% bile salt defects.

Notes:
*May be seen in adults.<ref name=pmid21325763>{{Cite journal | last1 = Hayashi | first1 = H. | last2 = Narita | first2 = R. | last3 = Hiura | first3 = M. | last4 = Abe | first4 = S. | last5 = Tabaru | first5 = A. | last6 = Tanimoto | first6 = A. | last7 = Sasaguri | first7 = Y. | last8 = Harada | first8 = M. | title = A case of adult autoimmune hepatitis with histological features of giant cell hepatitis. | journal = Intern Med | volume = 50 | issue = 4 | pages = 315-9 | month = | year = 2011 | doi = | PMID = 21325763 }}</ref>
*Reported association with [[subdural hematoma]].<ref name=pmid21331818>{{Cite journal | last1 = Guddat | first1 = SS. | last2 = Ehrlich | first2 = E. | last3 = Martin | first3 = H. | last4 = Tsokos | first4 = M. | title = Fatal spontaneous subdural bleeding due to neonatal giant cell hepatitis: a rare differential diagnosis of shaken baby syndrome. | journal = Forensic Sci Med Pathol | volume = 7 | issue = 3 | pages = 294-7 | month = Sep | year = 2011 | doi = 10.1007/s12024-011-9227-8 | PMID = 21331818 }}</ref>

===Microscopic===
Features:<ref name=pmid20871223>{{Cite journal | last1 = Torbenson | first1 = M. | last2 = Hart | first2 = J. | last3 = Westerhoff | first3 = M. | last4 = Azzam | first4 = RK. | last5 = Elgendi | first5 = A. | last6 = Mziray-Andrew | first6 = HC. | last7 = Kim | first7 = GE. | last8 = Scheimann | first8 = A. | title = Neonatal giant cell hepatitis: histological and etiological findings. | journal = Am J Surg Pathol | volume = 34 | issue = 10 | pages = 1498-503 | month = Oct | year = 2010 | doi = 10.1097/PAS.0b013e3181f069ab | PMID = 20871223 }}</ref>
*Giant hepatocytes with multiple nuclei - '''key feature'''.
**Typically ~35% of hepatocytes affected.
*Minimal or absent inflammation portal and lobular inflammation.
*Lobular cholestasis.

====Images====
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090423115718994 GCH (surgicalpathologyatlas.com)].

==Biliary atresia==
===General===
*1/3 of neonatal cholestasis.<ref name=Ref_PCPBoD8|464>{{Ref PCPBoD8|464}}</ref>
*Etiology - various.
**Viral - possibly rotavirus.<ref name=pmid22123643>{{Cite journal | last1 = Hertel | first1 = PM. | last2 = Estes | first2 = MK. | title = Rotavirus and biliary atresia: can causation be proven? | journal = Curr Opin Gastroenterol | volume = 28 | issue = 1 | pages = 10-7 | month = Jan | year = 2012 | doi = 10.1097/MOG.0b013e32834c7ae4 | PMID = 22123643 }}</ref>
**Genetic/syndromic - several.<ref name=pmid20425482>{{Cite journal | last1 = Santos | first1 = JL. | last2 = Choquette | first2 = M. | last3 = Bezerra | first3 = JA. | title = Cholestatic liver disease in children. | journal = Curr Gastroenterol Rep | volume = 12 | issue = 1 | pages = 30-9 | month = Feb | year = 2010 | doi = 10.1007/s11894-009-0081-8 | PMID = 20425482 }}</ref>

===Microscopic===
Features:<ref name=Ref_PCPBoD8|464/>
*Bile duct proliferation.
*Portal tract edema.
*Portal tract fibrosis.

Image:
*[http://oac.med.jhmi.edu/pathconcepts/ShowImage.cfm?TutorialID=3&ConceptID=12&ImageID=244 Biliary atresia (jhmi.edu)].

=See also=
*[[Pediatric pathology]].
*[[Gastrointestinal pathology]].

=References=
{{Reflist|2}}

[[Category:Pediatric pathology]]

Pediatric gastrointestinal pathology

2018-04-12T12:29:32Z

Brigitte: /* Necrotizing enterocolitis */

This article deals with '''pediatric gastrointestinal pathology'''. An introduction to pediatric pathology is in the ''[[pediatric pathology]]'' article.

An overview of (adult) gastrointestinal pathology is in the ''[[gastrointestinal pathology]]'' article.

=Birth defects=
==Omphalocele==
===General===
Usually genetic (unlike [[gastroschisis]]) - associated with:<ref name=pmid20809116>{{Cite journal | last1 = Frolov | first1 = P. | last2 = Alali | first2 = J. | last3 = Klein | first3 = MD. | title = Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature. | journal = Pediatr Surg Int | volume = 26 | issue = 12 | pages = 1135-48 | month = Dec | year = 2010 | doi = 10.1007/s00383-010-2701-7 | PMID = 20809116 }}</ref>
*[[Trisomy 18]] (Edwards syndrome) ~ 6% have omphaloceles in a series of 85 cases.<ref name=pmid3191615>{{Cite journal | last1 = Moore | first1 = CA. | last2 = Harmon | first2 = JP. | last3 = Padilla | first3 = LM. | last4 = Castro | first4 = VB. | last5 = Weaver | first5 = DD. | title = Neural tube defects and omphalocele in trisomy 18. | journal = Clin Genet | volume = 34 | issue = 2 | pages = 98-103 | month = Aug | year = 1988 | doi = | PMID = 3191615 }}</ref>
*[[Beckwith-Wiedemann syndrome]].

Presentation:
*Increased AFP.

===Gross===
*Bowel outside of abdomen - covered by membrane/in a sac.

Image:
*[http://library.med.utah.edu/WebPath/PEDHTML/PED006.html Omphalocele (utah.edu)].

==Gastroschisis==
===General===
*Defect considered to be more severe than [[omphalocele]].
*Usually sporadic.

===Gross===
*Bowel outside of abdomen - individual loops of bowel are seen.

Image:
*[http://med.brown.edu/pedisurg/Brown/IBImages/AbdWallDefects/Gastroschisis%202.html Gastroschisis (brown.edu)].

=Luminal pathology=
==Esophageal atresia==
===General===
*Multifactoral.
*Often associated with other abnormalities.

Forms:<ref name=pmid17498283>{{Cite journal | last1 = Spitz | first1 = L. | title = Oesophageal atresia. | journal = Orphanet J Rare Dis | volume = 2 | issue = | pages = 24 | month = | year = 2007 | doi = 10.1186/1750-1172-2-24 | PMID = 17498283 }}</ref>
#Esophageal atresia with distal tracheoesophageal fistula - most common.
#Esophageal atresia without a fistula.

Note:
*The "H-type" tracheoeosphageal fistula is often lumped together with ''esophageal atresia''.<ref name=pmid17498283/>

Image:
*[http://commons.wikimedia.org/wiki/File:Atrezja.jpg Esophageal atresia (WC)].

==Abetalipoproteinemia==
*[[AKA]] ''Bassen-Kornzweig syndrome''.

===General===
*Rare genetic disorder.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/200100 http://www.ncbi.nlm.nih.gov/omim/200100]. Accessed on: 6 April 2011.</ref><ref>{{cite journal |author=Bassen FA, Kornzweig AL |title=Malformation of the erythrocytes in a case of atypical retinitis pigmentosa |journal=Blood |volume=5 |issue=4 |pages=381–87 |year=1950 |month=April |pmid=15411425 |doi= |url=}}</ref>
*GI-related symptoms similar to [[celiac disease]] - malabsorption.

Clinical features:<ref name=pmid24139731>{{Cite journal | last1 = Hammer | first1 = MB. | last2 = El Euch-Fayache | first2 = G. | last3 = Nehdi | first3 = H. | last4 = Feki | first4 = M. | last5 = Maamouri-Hicheri | first5 = W. | last6 = Hentati | first6 = F. | last7 = Amouri | first7 = R. | title = Clinical features and molecular genetics of two Tunisian families with abetalipoproteinemia. | journal = J Clin Neurosci | volume = 21 | issue = 2 | pages = 311-5 | month = Feb | year = 2014 | doi = 10.1016/j.jocn.2013.04.016 | PMID = 24139731 }}</ref>
*Failure to thrive.
*Pigmented retinopathy.

Blood work:<ref name=pmid24139731/>
*Cholesterol - low.
*Triglyceride - low.
*Apolipoprotein B - very low.

===Microscopic===
Features:
*Enterocytes have clear cytoplasm (due to lipid accumulation).

Notes:
*Have abnormal erythrocytes with a spiculated cell membranes ''acanthocyte'' - seen on blood films.

====Images====
<gallery>
Image:Abetalipoproteinemia_-_intermed_mag.jpg | Abetalipoproteinemia - intermed. mag. (WC)
Image:Abetalipoproteinemia_-_very_high_mag.jpg | Abetalipoproteinemia - very high mag. (WC)
</gallery>

==Microvillous inclusion disease==
*[[AKA]] ''Davidson disease''.
===General===
*Autosomal recessive inherited condition - due to mutation in ''MYO5B''.<ref name=pmid18724368>{{cite journal |author=Müller T, Hess MW, Schiefermeier N, ''et al.'' |title=MYO5B mutations cause microvillus inclusion disease and disrupt epithelial cell polarity |journal=Nat. Genet. |volume=40 |issue=10 |pages=1163–5 |year=2008 |month=October |pmid=18724368 |doi=10.1038/ng.225 |url=}}</ref>

===Microscopic===
Features:
*Flat mucosa; no villi.

Notes:
*Appearance similar to [[celiac sprue]]; however, usually lacks the intraepithelial lymphocytic infiltration characteristic of [[celiac sprue]].

Images:
*[http://path.upmc.edu/cases/case163.html Microvillous inclusion disease - several images (upmc.edu)].

===IHC===
*[[Carcinoembryonic antigen]] (CEA) +ve.<ref name=Ref_Sternberg4>{{Ref Sternberg4|}}</ref>

===EM===
*Diagnosis is dependent on [[electron microscopy]].<ref name=pmid11251929>{{cite journal |author=Kennea N, Norbury R, Anderson G, Tekay A |title=Congenital microvillous inclusion disease presenting as antenatal bowel obstruction |journal=Ultrasound Obstet Gynecol |volume=17 |issue=2 |pages=172–4 |year=2001 |pmid=11251929 |doi=10.1046/j.1469-0705.2001.00211.x}}</ref>

Images:
*[http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=1398 MID (gfmer.ch)].

==Cystic fibrosis==
{{Main|Cystic fibrosis}}

===General===
*Genetic.
*May lead to [[meconium ileus]].

===Microscopic===
Features - large bowel:<ref name=pmid710839>{{cite journal |author=Neutra MR, Trier JS |title=Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture |journal=Gastroenterology |volume=75 |issue=4 |pages=701–10 |year=1978 |month=October |pmid=710839 |doi= |url=}}</ref>
*Crypt enlargement.

Notes:
*''Not'' intracellular and extracellular accumulation of mucus. (?)

==Colonic aganglionosis==
*[[AKA]] ''Hirschsprung disease''.
===General===
*Genetic disorder:<ref>{{OMIM|142623}}</ref>
**5-10% familial; RET gene most commonly mutated.
**Several genes involved.
**Inheritance pattern variable.
*Treatment: surgery (''Swenson's procedure'' or ''Duhamel procedure'').<ref name=pmid6649901>{{Cite journal | last1 = Okasora | first1 = T. | last2 = Okamoto | first2 = E. | last3 = Kuwata | first3 = K. | last4 = Toyosaka | first4 = A. | last5 = Ohashi | first5 = S. | last6 = Ueki | first6 = S. | title = Serum and erythrocyte acetylcholine esterase in Hirschsprung's disease. | journal = Z Kinderchir | volume = 38 | issue = 5 | pages = 298-300 | month = Oct | year = 1983 | doi = 10.1055/s-2008-1059992 | PMID = 6649901 }}</ref>

Pathology:
*Failure of neural crest cell migration
**Parasympathetic ganglion cells in submucosal plexus (Meissner plexus) and myenteric plexus (Auerbach plexus) - absent.<ref name=pmid17139897>{{Cite journal | last1 = Vorobyov | first1 = GI. | last2 = Achkasov | first2 = SI. | last3 = Biryukov | first3 = OM. | title = Hirschsprung's disease in adults. | journal = Acta Chir Iugosl | volume = 53 | issue = 2 | pages = 113-6 | month = | year = 2006 | doi = | PMID = 17139897 }}</ref>

Notes:
*Most common reason for litigation in paediatric pathology.<ref>Taylor, G. 19 January 2011.</ref>

===Gross===
Features:
*Dilated bowel; stuffed sausage-look.

Classification:
*Short-segment (75-80%): Rectum, distal sigmoid
*Long-segment HD (10-20%): Beyond splenic flexure
*Total colonic aganglionosis (5-15%): Entire colon.<ref name=pmid25395999>{{Cite web | last = | first = | title = Diagnosis of Hirschsprung's disease with particular emphasis on histopathology. A systematic review of current literature | url = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/ | publisher = | date = | accessdate = 19 August 2017 }}</ref>

**[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/figure/F0001/ Classification of Hirschsprung's disease according to the aganglionic segment length (https://www.ncbi.nlm.nih.gov)]

Image:
*[http://www.pathology.pitt.edu/lectures/gi/colon-a/03.htm Hirschsprung disease (pathology.pitt.edu)].

===Microscopic===
Features:
*Ganglion cells missing in submucosal plexus and myenteric plexus.
**Increasing ganglia proximally into transition zone
*Hypertrophy of neural plexuses.
**Many nerve trunks > 40 μm
*Abnormal submucosal blood vessels may be seen
*+/-Submucosal fibrosis.

Image:
*[http://pathology.mc.duke.edu/research/Histo_course/myent_plexus.jpg Normal myenteric plexus (duke.edu)].<ref>URL: [http://pathology.mc.duke.edu/research/PTH225.html http://pathology.mc.duke.edu/research/PTH225.html]. Accessed on: 11 January 2011.</ref>

===Stains===
*Acetylcholinesterase - marks the abundant, disorganized, nerve fibers.

Image:
*[http://commons.wikimedia.org/wiki/File:Hirschsprung_acetylcholine.jpg Hirschsprung - acetylcholinesterase (WC)].

===IHC===
Features:<ref name=pmid1640323>{{Cite journal | last1 = Luider | first1 = TM. | last2 = van Dommelen | first2 = MW. | last3 = Tibboel | first3 = D. | last4 = Meijers | first4 = JH. | last5 = Ten Kate | first5 = FJ. | last6 = Trojanowski | first6 = JQ. | last7 = Molenaar | first7 = JC. | title = Differences in phosphorylation state of neurofilament proteins in ganglionic and aganglionic bowel segments of children with Hirschsprung's disease. | journal = J Pediatr Surg | volume = 27 | issue = 7 | pages = 815-9 | month = Jul | year = 1992 | doi = | PMID = 1640323 }}</ref>
*NF-M (neurofilament middle) - highlight hypertrophic nerve fascicules
*NF-H (neurofilament high) - highlight hypertrophic nerve fascicules.
*Tau ~ highlights ganglion cells (which are absent in segments affected by Hirschsprung).<ref name=pmid8229560>{{Cite journal | last1 = Deguchi | first1 = E. | last2 = Iwai | first2 = N. | last3 = Goto | first3 = Y. | last4 = Yanagihara | first4 = J. | last5 = Fushiki | first5 = S. | title = An immunohistochemical study of neurofilament and microtubule-associated Tau protein in the enteric innervation in Hirschsprung's disease. | journal = J Pediatr Surg | volume = 28 | issue = 7 | pages = 886-90 | month = Jul | year = 1993 | doi = | PMID = 8229560 }}</ref>
**Nerve fibres +ve control.

Others<ref>{{Cite book | last1 = Lin | first1 = Fan | last2 = Prichard | first2 = Jeffrey | title = Handbook of practical immunohistochemistry : frequently asked question | date = | publisher = | location = | isbn = 9781493915774 | pages = }}</ref>
:
*Calretinin ~ -ve, Usually negative in hypertrophied nerve fibers in Hirschsprung’s disease, superior to acetylcholinesterase.
*NSE ~ -ve, Highlights ganglion cells to exclude Hirschsprung’s disease; specific but not very sensitive.

==Meconium ileus==
===General===
*Classically due to ''[[cystic fibrosis]]''.
*May lead to ''[[meconium peritonitis]]''.
*Can be mimicked by [[CMV]] infection.<ref>{{cite journal |author=Déchelotte PJ, Mulliez NM, Bouvier RJ, Vanlieféringhen PC, Lémery DJ |title=Pseudo-meconium ileus due to cytomegalovirus infection: a report of three cases |journal=Pediatr Pathol |volume=12 |issue=1 |pages=73–82 |year=1992 |pmid=1313975 |doi= |url=}}</ref>

===Gross===
Features:
*Thick.
**High viscosity.
*Green.

====Image====
*[http://library.med.utah.edu/WebPath/jpeg3/PERI175.jpg Meconium ileus (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html]. Accessed on: 3 December 2011.</ref>

===Microscopic===
Features:
*Meconium-laden macrophages. (???)

==Meconium peritonitis==
===General===
*May be due to a number of causes:
**Aganglionosis ([[Hirschsprung disease]]).
**[[Meconium ileus]].

===Microscopic===
Features:
*Brown granular material - '''key feature'''.
*+/-Multinucleated giant cells.
*Inflammatory infiltrate (PMNs, lymphocytes, plasma cells).

Image:
*[http://www.pathologyoutlines.com/caseofweek/case2008106image2.jpg Meconium peritonitis - gross (pathologyoutlines.com)].

==Necrotizing enterocolitis==
*Abbreviated ''NEC''.
===General===
*Disease primarily of premature babies.
*Diagnosed by imaging.

Note:
*''Enterocolitis'' = inflammation of [[small bowel]] and [[colon]].<ref>URL: [http://medical-dictionary.thefreedictionary.com/enterocolitis http://medical-dictionary.thefreedictionary.com/enterocolitis]. Accessed on: 10 October 2011.</ref>
**''Necrotizing enteritis'' = small bowel only.

===Microscopic===
Features:
*Large spaces.

DDx:
*[[Pneumatosis intestinalis]].

Images:
*[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)].
*[https://library.med.utah.edu/WebPath/PEDHTML/PED045.html].

==Autoimmune enteropathy==
{{Main|Autoimmune enteropathy}}

=Pancreas=
{{Main|Pancreas}}
==Pancreatic islet cell hyperplasia==
===General===
*Associated with maternal [[diabetes]].

===Microscopic===
Features:
*Marked size variation of pancreatic islets.
**Normal islets ~ 150 micrometers (diameter). Hyperplastic islets - up to ~500 micrometers (diameter).

Image:
*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].

=Liver=
{{Main|Liver pathology}}
==Giant cell hepatitis==
*[[AKA]] ''neonatal giant cell hepatitis'', abbreviated ''NGCH''.
===General===
*Rare.

Etiology:
*Unknown - possibly viral, autoimmune and/or drugs.<ref name=pmid21043007>{{Cite journal | last1 = Hartl | first1 = J. | last2 = Buettner | first2 = R. | last3 = Rockmann | first3 = F. | last4 = Farkas | first4 = S. | last5 = Holstege | first5 = A. | last6 = Vogel | first6 = C. | last7 = Schnitzbauer | first7 = A. | last8 = Schlitt | first8 = HJ. | last9 = Schoelmerich | first9 = J. | title = Giant cell hepatitis: an unusual cause of fulminant liver failure. | journal = Z Gastroenterol | volume = 48 | issue = 11 | pages = 1293-6 | month = Nov | year = 2010 | doi = 10.1055/s-0029-1245476 | PMID = 21043007 }}</ref>
*One large series suggests that, in the neonatal population, with follow-up the causes are:
**~49% idiopathic.
**~16% pan-hypopituitarism.
**~8% biliary atresia.
**~6% Alagille syndrome
**~6% bile salt defects.

Notes:
*May be seen in adults.<ref name=pmid21325763>{{Cite journal | last1 = Hayashi | first1 = H. | last2 = Narita | first2 = R. | last3 = Hiura | first3 = M. | last4 = Abe | first4 = S. | last5 = Tabaru | first5 = A. | last6 = Tanimoto | first6 = A. | last7 = Sasaguri | first7 = Y. | last8 = Harada | first8 = M. | title = A case of adult autoimmune hepatitis with histological features of giant cell hepatitis. | journal = Intern Med | volume = 50 | issue = 4 | pages = 315-9 | month = | year = 2011 | doi = | PMID = 21325763 }}</ref>
*Reported association with [[subdural hematoma]].<ref name=pmid21331818>{{Cite journal | last1 = Guddat | first1 = SS. | last2 = Ehrlich | first2 = E. | last3 = Martin | first3 = H. | last4 = Tsokos | first4 = M. | title = Fatal spontaneous subdural bleeding due to neonatal giant cell hepatitis: a rare differential diagnosis of shaken baby syndrome. | journal = Forensic Sci Med Pathol | volume = 7 | issue = 3 | pages = 294-7 | month = Sep | year = 2011 | doi = 10.1007/s12024-011-9227-8 | PMID = 21331818 }}</ref>

===Microscopic===
Features:<ref name=pmid20871223>{{Cite journal | last1 = Torbenson | first1 = M. | last2 = Hart | first2 = J. | last3 = Westerhoff | first3 = M. | last4 = Azzam | first4 = RK. | last5 = Elgendi | first5 = A. | last6 = Mziray-Andrew | first6 = HC. | last7 = Kim | first7 = GE. | last8 = Scheimann | first8 = A. | title = Neonatal giant cell hepatitis: histological and etiological findings. | journal = Am J Surg Pathol | volume = 34 | issue = 10 | pages = 1498-503 | month = Oct | year = 2010 | doi = 10.1097/PAS.0b013e3181f069ab | PMID = 20871223 }}</ref>
*Giant hepatocytes with multiple nuclei - '''key feature'''.
**Typically ~35% of hepatocytes affected.
*Minimal or absent inflammation portal and lobular inflammation.
*Lobular cholestasis.

====Images====
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090423115718994 GCH (surgicalpathologyatlas.com)].

==Biliary atresia==
===General===
*1/3 of neonatal cholestasis.<ref name=Ref_PCPBoD8|464>{{Ref PCPBoD8|464}}</ref>
*Etiology - various.
**Viral - possibly rotavirus.<ref name=pmid22123643>{{Cite journal | last1 = Hertel | first1 = PM. | last2 = Estes | first2 = MK. | title = Rotavirus and biliary atresia: can causation be proven? | journal = Curr Opin Gastroenterol | volume = 28 | issue = 1 | pages = 10-7 | month = Jan | year = 2012 | doi = 10.1097/MOG.0b013e32834c7ae4 | PMID = 22123643 }}</ref>
**Genetic/syndromic - several.<ref name=pmid20425482>{{Cite journal | last1 = Santos | first1 = JL. | last2 = Choquette | first2 = M. | last3 = Bezerra | first3 = JA. | title = Cholestatic liver disease in children. | journal = Curr Gastroenterol Rep | volume = 12 | issue = 1 | pages = 30-9 | month = Feb | year = 2010 | doi = 10.1007/s11894-009-0081-8 | PMID = 20425482 }}</ref>

===Microscopic===
Features:<ref name=Ref_PCPBoD8|464/>
*Bile duct proliferation.
*Portal tract edema.
*Portal tract fibrosis.

Image:
*[http://oac.med.jhmi.edu/pathconcepts/ShowImage.cfm?TutorialID=3&ConceptID=12&ImageID=244 Biliary atresia (jhmi.edu)].

=See also=
*[[Pediatric pathology]].
*[[Gastrointestinal pathology]].

=References=
{{Reflist|2}}

[[Category:Pediatric pathology]]

Pediatric gastrointestinal pathology

2018-04-12T12:25:10Z

Brigitte: /* Microscopic */

This article deals with '''pediatric gastrointestinal pathology'''. An introduction to pediatric pathology is in the ''[[pediatric pathology]]'' article.

An overview of (adult) gastrointestinal pathology is in the ''[[gastrointestinal pathology]]'' article.

=Birth defects=
==Omphalocele==
===General===
Usually genetic (unlike [[gastroschisis]]) - associated with:<ref name=pmid20809116>{{Cite journal | last1 = Frolov | first1 = P. | last2 = Alali | first2 = J. | last3 = Klein | first3 = MD. | title = Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature. | journal = Pediatr Surg Int | volume = 26 | issue = 12 | pages = 1135-48 | month = Dec | year = 2010 | doi = 10.1007/s00383-010-2701-7 | PMID = 20809116 }}</ref>
*[[Trisomy 18]] (Edwards syndrome) ~ 6% have omphaloceles in a series of 85 cases.<ref name=pmid3191615>{{Cite journal | last1 = Moore | first1 = CA. | last2 = Harmon | first2 = JP. | last3 = Padilla | first3 = LM. | last4 = Castro | first4 = VB. | last5 = Weaver | first5 = DD. | title = Neural tube defects and omphalocele in trisomy 18. | journal = Clin Genet | volume = 34 | issue = 2 | pages = 98-103 | month = Aug | year = 1988 | doi = | PMID = 3191615 }}</ref>
*[[Beckwith-Wiedemann syndrome]].

Presentation:
*Increased AFP.

===Gross===
*Bowel outside of abdomen - covered by membrane/in a sac.

Image:
*[http://library.med.utah.edu/WebPath/PEDHTML/PED006.html Omphalocele (utah.edu)].

==Gastroschisis==
===General===
*Defect considered to be more severe than [[omphalocele]].
*Usually sporadic.

===Gross===
*Bowel outside of abdomen - individual loops of bowel are seen.

Image:
*[http://med.brown.edu/pedisurg/Brown/IBImages/AbdWallDefects/Gastroschisis%202.html Gastroschisis (brown.edu)].

=Luminal pathology=
==Esophageal atresia==
===General===
*Multifactoral.
*Often associated with other abnormalities.

Forms:<ref name=pmid17498283>{{Cite journal | last1 = Spitz | first1 = L. | title = Oesophageal atresia. | journal = Orphanet J Rare Dis | volume = 2 | issue = | pages = 24 | month = | year = 2007 | doi = 10.1186/1750-1172-2-24 | PMID = 17498283 }}</ref>
#Esophageal atresia with distal tracheoesophageal fistula - most common.
#Esophageal atresia without a fistula.

Note:
*The "H-type" tracheoeosphageal fistula is often lumped together with ''esophageal atresia''.<ref name=pmid17498283/>

Image:
*[http://commons.wikimedia.org/wiki/File:Atrezja.jpg Esophageal atresia (WC)].

==Abetalipoproteinemia==
*[[AKA]] ''Bassen-Kornzweig syndrome''.

===General===
*Rare genetic disorder.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/200100 http://www.ncbi.nlm.nih.gov/omim/200100]. Accessed on: 6 April 2011.</ref><ref>{{cite journal |author=Bassen FA, Kornzweig AL |title=Malformation of the erythrocytes in a case of atypical retinitis pigmentosa |journal=Blood |volume=5 |issue=4 |pages=381–87 |year=1950 |month=April |pmid=15411425 |doi= |url=}}</ref>
*GI-related symptoms similar to [[celiac disease]] - malabsorption.

Clinical features:<ref name=pmid24139731>{{Cite journal | last1 = Hammer | first1 = MB. | last2 = El Euch-Fayache | first2 = G. | last3 = Nehdi | first3 = H. | last4 = Feki | first4 = M. | last5 = Maamouri-Hicheri | first5 = W. | last6 = Hentati | first6 = F. | last7 = Amouri | first7 = R. | title = Clinical features and molecular genetics of two Tunisian families with abetalipoproteinemia. | journal = J Clin Neurosci | volume = 21 | issue = 2 | pages = 311-5 | month = Feb | year = 2014 | doi = 10.1016/j.jocn.2013.04.016 | PMID = 24139731 }}</ref>
*Failure to thrive.
*Pigmented retinopathy.

Blood work:<ref name=pmid24139731/>
*Cholesterol - low.
*Triglyceride - low.
*Apolipoprotein B - very low.

===Microscopic===
Features:
*Enterocytes have clear cytoplasm (due to lipid accumulation).

Notes:
*Have abnormal erythrocytes with a spiculated cell membranes ''acanthocyte'' - seen on blood films.

====Images====
<gallery>
Image:Abetalipoproteinemia_-_intermed_mag.jpg | Abetalipoproteinemia - intermed. mag. (WC)
Image:Abetalipoproteinemia_-_very_high_mag.jpg | Abetalipoproteinemia - very high mag. (WC)
</gallery>

==Microvillous inclusion disease==
*[[AKA]] ''Davidson disease''.
===General===
*Autosomal recessive inherited condition - due to mutation in ''MYO5B''.<ref name=pmid18724368>{{cite journal |author=Müller T, Hess MW, Schiefermeier N, ''et al.'' |title=MYO5B mutations cause microvillus inclusion disease and disrupt epithelial cell polarity |journal=Nat. Genet. |volume=40 |issue=10 |pages=1163–5 |year=2008 |month=October |pmid=18724368 |doi=10.1038/ng.225 |url=}}</ref>

===Microscopic===
Features:
*Flat mucosa; no villi.

Notes:
*Appearance similar to [[celiac sprue]]; however, usually lacks the intraepithelial lymphocytic infiltration characteristic of [[celiac sprue]].

Images:
*[http://path.upmc.edu/cases/case163.html Microvillous inclusion disease - several images (upmc.edu)].

===IHC===
*[[Carcinoembryonic antigen]] (CEA) +ve.<ref name=Ref_Sternberg4>{{Ref Sternberg4|}}</ref>

===EM===
*Diagnosis is dependent on [[electron microscopy]].<ref name=pmid11251929>{{cite journal |author=Kennea N, Norbury R, Anderson G, Tekay A |title=Congenital microvillous inclusion disease presenting as antenatal bowel obstruction |journal=Ultrasound Obstet Gynecol |volume=17 |issue=2 |pages=172–4 |year=2001 |pmid=11251929 |doi=10.1046/j.1469-0705.2001.00211.x}}</ref>

Images:
*[http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=1398 MID (gfmer.ch)].

==Cystic fibrosis==
{{Main|Cystic fibrosis}}

===General===
*Genetic.
*May lead to [[meconium ileus]].

===Microscopic===
Features - large bowel:<ref name=pmid710839>{{cite journal |author=Neutra MR, Trier JS |title=Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture |journal=Gastroenterology |volume=75 |issue=4 |pages=701–10 |year=1978 |month=October |pmid=710839 |doi= |url=}}</ref>
*Crypt enlargement.

Notes:
*''Not'' intracellular and extracellular accumulation of mucus. (?)

==Colonic aganglionosis==
*[[AKA]] ''Hirschsprung disease''.
===General===
*Genetic disorder:<ref>{{OMIM|142623}}</ref>
**5-10% familial; RET gene most commonly mutated.
**Several genes involved.
**Inheritance pattern variable.
*Treatment: surgery (''Swenson's procedure'' or ''Duhamel procedure'').<ref name=pmid6649901>{{Cite journal | last1 = Okasora | first1 = T. | last2 = Okamoto | first2 = E. | last3 = Kuwata | first3 = K. | last4 = Toyosaka | first4 = A. | last5 = Ohashi | first5 = S. | last6 = Ueki | first6 = S. | title = Serum and erythrocyte acetylcholine esterase in Hirschsprung's disease. | journal = Z Kinderchir | volume = 38 | issue = 5 | pages = 298-300 | month = Oct | year = 1983 | doi = 10.1055/s-2008-1059992 | PMID = 6649901 }}</ref>

Pathology:
*Failure of neural crest cell migration
**Parasympathetic ganglion cells in submucosal plexus (Meissner plexus) and myenteric plexus (Auerbach plexus) - absent.<ref name=pmid17139897>{{Cite journal | last1 = Vorobyov | first1 = GI. | last2 = Achkasov | first2 = SI. | last3 = Biryukov | first3 = OM. | title = Hirschsprung's disease in adults. | journal = Acta Chir Iugosl | volume = 53 | issue = 2 | pages = 113-6 | month = | year = 2006 | doi = | PMID = 17139897 }}</ref>

Notes:
*Most common reason for litigation in paediatric pathology.<ref>Taylor, G. 19 January 2011.</ref>

===Gross===
Features:
*Dilated bowel; stuffed sausage-look.

Classification:
*Short-segment (75-80%): Rectum, distal sigmoid
*Long-segment HD (10-20%): Beyond splenic flexure
*Total colonic aganglionosis (5-15%): Entire colon.<ref name=pmid25395999>{{Cite web | last = | first = | title = Diagnosis of Hirschsprung's disease with particular emphasis on histopathology. A systematic review of current literature | url = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/ | publisher = | date = | accessdate = 19 August 2017 }}</ref>

**[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223113/figure/F0001/ Classification of Hirschsprung's disease according to the aganglionic segment length (https://www.ncbi.nlm.nih.gov)]

Image:
*[http://www.pathology.pitt.edu/lectures/gi/colon-a/03.htm Hirschsprung disease (pathology.pitt.edu)].

===Microscopic===
Features:
*Ganglion cells missing in submucosal plexus and myenteric plexus.
**Increasing ganglia proximally into transition zone
*Hypertrophy of neural plexuses.
**Many nerve trunks > 40 μm
*Abnormal submucosal blood vessels may be seen
*+/-Submucosal fibrosis.

Image:
*[http://pathology.mc.duke.edu/research/Histo_course/myent_plexus.jpg Normal myenteric plexus (duke.edu)].<ref>URL: [http://pathology.mc.duke.edu/research/PTH225.html http://pathology.mc.duke.edu/research/PTH225.html]. Accessed on: 11 January 2011.</ref>

===Stains===
*Acetylcholinesterase - marks the abundant, disorganized, nerve fibers.

Image:
*[http://commons.wikimedia.org/wiki/File:Hirschsprung_acetylcholine.jpg Hirschsprung - acetylcholinesterase (WC)].

===IHC===
Features:<ref name=pmid1640323>{{Cite journal | last1 = Luider | first1 = TM. | last2 = van Dommelen | first2 = MW. | last3 = Tibboel | first3 = D. | last4 = Meijers | first4 = JH. | last5 = Ten Kate | first5 = FJ. | last6 = Trojanowski | first6 = JQ. | last7 = Molenaar | first7 = JC. | title = Differences in phosphorylation state of neurofilament proteins in ganglionic and aganglionic bowel segments of children with Hirschsprung's disease. | journal = J Pediatr Surg | volume = 27 | issue = 7 | pages = 815-9 | month = Jul | year = 1992 | doi = | PMID = 1640323 }}</ref>
*NF-M (neurofilament middle) - highlight hypertrophic nerve fascicules
*NF-H (neurofilament high) - highlight hypertrophic nerve fascicules.
*Tau ~ highlights ganglion cells (which are absent in segments affected by Hirschsprung).<ref name=pmid8229560>{{Cite journal | last1 = Deguchi | first1 = E. | last2 = Iwai | first2 = N. | last3 = Goto | first3 = Y. | last4 = Yanagihara | first4 = J. | last5 = Fushiki | first5 = S. | title = An immunohistochemical study of neurofilament and microtubule-associated Tau protein in the enteric innervation in Hirschsprung's disease. | journal = J Pediatr Surg | volume = 28 | issue = 7 | pages = 886-90 | month = Jul | year = 1993 | doi = | PMID = 8229560 }}</ref>
**Nerve fibres +ve control.

Others<ref>{{Cite book | last1 = Lin | first1 = Fan | last2 = Prichard | first2 = Jeffrey | title = Handbook of practical immunohistochemistry : frequently asked question | date = | publisher = | location = | isbn = 9781493915774 | pages = }}</ref>
:
*Calretinin ~ -ve, Usually negative in hypertrophied nerve fibers in Hirschsprung’s disease, superior to acetylcholinesterase.
*NSE ~ -ve, Highlights ganglion cells to exclude Hirschsprung’s disease; specific but not very sensitive.

==Meconium ileus==
===General===
*Classically due to ''[[cystic fibrosis]]''.
*May lead to ''[[meconium peritonitis]]''.
*Can be mimicked by [[CMV]] infection.<ref>{{cite journal |author=Déchelotte PJ, Mulliez NM, Bouvier RJ, Vanlieféringhen PC, Lémery DJ |title=Pseudo-meconium ileus due to cytomegalovirus infection: a report of three cases |journal=Pediatr Pathol |volume=12 |issue=1 |pages=73–82 |year=1992 |pmid=1313975 |doi= |url=}}</ref>

===Gross===
Features:
*Thick.
**High viscosity.
*Green.

====Image====
*[http://library.med.utah.edu/WebPath/jpeg3/PERI175.jpg Meconium ileus (med.utah.edu)].<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pdfrm.html]. Accessed on: 3 December 2011.</ref>

===Microscopic===
Features:
*Meconium-laden macrophages. (???)

==Meconium peritonitis==
===General===
*May be due to a number of causes:
**Aganglionosis ([[Hirschsprung disease]]).
**[[Meconium ileus]].

===Microscopic===
Features:
*Brown granular material - '''key feature'''.
*+/-Multinucleated giant cells.
*Inflammatory infiltrate (PMNs, lymphocytes, plasma cells).

Image:
*[http://www.pathologyoutlines.com/caseofweek/case2008106image2.jpg Meconium peritonitis - gross (pathologyoutlines.com)].

==Necrotizing enterocolitis==
*Abbreviated ''NEC''.
===General===
*Disease primarily of premature babies.
*Diagnosed by imaging.

Note:
*''Enterocolitis'' = inflammation of [[small bowel]] and [[colon]].<ref>URL: [http://medical-dictionary.thefreedictionary.com/enterocolitis http://medical-dictionary.thefreedictionary.com/enterocolitis]. Accessed on: 10 October 2011.</ref>
**''Necrotizing enteritis'' = small bowel only.

===Microscopic===
Features:
*Large spaces.

DDx:
*[[Pneumatosis intestinalis]].

Images:
*[http://en.wikipedia.org/wiki/File:Neonatal_necrotizing_enterocolitis,_gross_pathology_20G0021_lores.jpg NEC - gross (WP)].

==Autoimmune enteropathy==
{{Main|Autoimmune enteropathy}}

=Pancreas=
{{Main|Pancreas}}
==Pancreatic islet cell hyperplasia==
===General===
*Associated with maternal [[diabetes]].

===Microscopic===
Features:
*Marked size variation of pancreatic islets.
**Normal islets ~ 150 micrometers (diameter). Hyperplastic islets - up to ~500 micrometers (diameter).

Image:
*[http://eulep.pdn.cam.ac.uk/pathbase2/Search_Pathbase/factsheet.php?image_number=3297 Islet cell hyperplasia - mouse (cam.ac.uk)].

=Liver=
{{Main|Liver pathology}}
==Giant cell hepatitis==
*[[AKA]] ''neonatal giant cell hepatitis'', abbreviated ''NGCH''.
===General===
*Rare.

Etiology:
*Unknown - possibly viral, autoimmune and/or drugs.<ref name=pmid21043007>{{Cite journal | last1 = Hartl | first1 = J. | last2 = Buettner | first2 = R. | last3 = Rockmann | first3 = F. | last4 = Farkas | first4 = S. | last5 = Holstege | first5 = A. | last6 = Vogel | first6 = C. | last7 = Schnitzbauer | first7 = A. | last8 = Schlitt | first8 = HJ. | last9 = Schoelmerich | first9 = J. | title = Giant cell hepatitis: an unusual cause of fulminant liver failure. | journal = Z Gastroenterol | volume = 48 | issue = 11 | pages = 1293-6 | month = Nov | year = 2010 | doi = 10.1055/s-0029-1245476 | PMID = 21043007 }}</ref>
*One large series suggests that, in the neonatal population, with follow-up the causes are:
**~49% idiopathic.
**~16% pan-hypopituitarism.
**~8% biliary atresia.
**~6% Alagille syndrome
**~6% bile salt defects.

Notes:
*May be seen in adults.<ref name=pmid21325763>{{Cite journal | last1 = Hayashi | first1 = H. | last2 = Narita | first2 = R. | last3 = Hiura | first3 = M. | last4 = Abe | first4 = S. | last5 = Tabaru | first5 = A. | last6 = Tanimoto | first6 = A. | last7 = Sasaguri | first7 = Y. | last8 = Harada | first8 = M. | title = A case of adult autoimmune hepatitis with histological features of giant cell hepatitis. | journal = Intern Med | volume = 50 | issue = 4 | pages = 315-9 | month = | year = 2011 | doi = | PMID = 21325763 }}</ref>
*Reported association with [[subdural hematoma]].<ref name=pmid21331818>{{Cite journal | last1 = Guddat | first1 = SS. | last2 = Ehrlich | first2 = E. | last3 = Martin | first3 = H. | last4 = Tsokos | first4 = M. | title = Fatal spontaneous subdural bleeding due to neonatal giant cell hepatitis: a rare differential diagnosis of shaken baby syndrome. | journal = Forensic Sci Med Pathol | volume = 7 | issue = 3 | pages = 294-7 | month = Sep | year = 2011 | doi = 10.1007/s12024-011-9227-8 | PMID = 21331818 }}</ref>

===Microscopic===
Features:<ref name=pmid20871223>{{Cite journal | last1 = Torbenson | first1 = M. | last2 = Hart | first2 = J. | last3 = Westerhoff | first3 = M. | last4 = Azzam | first4 = RK. | last5 = Elgendi | first5 = A. | last6 = Mziray-Andrew | first6 = HC. | last7 = Kim | first7 = GE. | last8 = Scheimann | first8 = A. | title = Neonatal giant cell hepatitis: histological and etiological findings. | journal = Am J Surg Pathol | volume = 34 | issue = 10 | pages = 1498-503 | month = Oct | year = 2010 | doi = 10.1097/PAS.0b013e3181f069ab | PMID = 20871223 }}</ref>
*Giant hepatocytes with multiple nuclei - '''key feature'''.
**Typically ~35% of hepatocytes affected.
*Minimal or absent inflammation portal and lobular inflammation.
*Lobular cholestasis.

====Images====
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090423115718994 GCH (surgicalpathologyatlas.com)].

==Biliary atresia==
===General===
*1/3 of neonatal cholestasis.<ref name=Ref_PCPBoD8|464>{{Ref PCPBoD8|464}}</ref>
*Etiology - various.
**Viral - possibly rotavirus.<ref name=pmid22123643>{{Cite journal | last1 = Hertel | first1 = PM. | last2 = Estes | first2 = MK. | title = Rotavirus and biliary atresia: can causation be proven? | journal = Curr Opin Gastroenterol | volume = 28 | issue = 1 | pages = 10-7 | month = Jan | year = 2012 | doi = 10.1097/MOG.0b013e32834c7ae4 | PMID = 22123643 }}</ref>
**Genetic/syndromic - several.<ref name=pmid20425482>{{Cite journal | last1 = Santos | first1 = JL. | last2 = Choquette | first2 = M. | last3 = Bezerra | first3 = JA. | title = Cholestatic liver disease in children. | journal = Curr Gastroenterol Rep | volume = 12 | issue = 1 | pages = 30-9 | month = Feb | year = 2010 | doi = 10.1007/s11894-009-0081-8 | PMID = 20425482 }}</ref>

===Microscopic===
Features:<ref name=Ref_PCPBoD8|464/>
*Bile duct proliferation.
*Portal tract edema.
*Portal tract fibrosis.

Image:
*[http://oac.med.jhmi.edu/pathconcepts/ShowImage.cfm?TutorialID=3&ConceptID=12&ImageID=244 Biliary atresia (jhmi.edu)].

=See also=
*[[Pediatric pathology]].
*[[Gastrointestinal pathology]].

=References=
{{Reflist|2}}

[[Category:Pediatric pathology]]

Meckel diverticulum

2018-04-08T21:37:12Z

Brigitte: /* Images */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image =
| Width =
| Caption =
| Synonyms =
| Micro = small bowel mucosa, +/-gastric mucosa (foveolar epithelium, oxyntic mucosa), +/-pancreatic epithelium
| Subtypes =
| LMDDx =
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross = small bowel outpouching on antemesenteric aspect ~5 cm long, ~60 cm from the [[ileocecal valve]]
| Grossing =
| Staging =
| Site = [[small intestine]]
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs =
| Symptoms = abdominal pain
| Prevalence = uncommon ~2% of population
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = benign
| Other =
| ClinDDx = [[acute appendicitis]], other causes of abdominal pain
| Tx = surgical removal
}}
'''Meckel diverticulum''' (also '''Meckel's diverticulum'''), is congenital structure of the distal [[small bowel]] that occasionally gets inflamed and may present with [[acute appendicitis]]-like symptoms.

==General==
*Most common congenital anomaly of the gastrointestinal tract.<ref name=pmid15026601>{{Cite journal | last1 = Levy | first1 = AD. | last2 = Hobbs | first2 = CM. | title = From the archives of the AFIP. Meckel diverticulum: radiologic features with pathologic Correlation. | journal = Radiographics | volume = 24 | issue = 2 | pages = 565-87 | month = | year = | doi = 10.1148/rg.242035187 | PMID = 15026601 }}</ref>
**Remnant of the ''omphalomesenteric duct'' - a connection of the yolk sac and midgut.

The rule of 2s:
*2 feet from the terminal ileum
*2% of the population
*2% symptomatic.
*2 inches long.
*2 year old.
*2 types of epithelium - gastric and pancreatic.

Main clinical DDx of a symptomatic Meckel diverticulum:
*[[Appendicitis]].

==Gross==
*Antimesenteric attachement, i.e. a ''Meckel's diverticulum'' hangs off the side opposite of the mesentery.

===Image===
<gallery>
Image:Meckel%27s_Diverticulum_AFIP.jpg | Meckel diverticulum. (AFIP/WC)
</gallery>

==Microscopic==
Features:<ref name=pmid15026601/>
*Small bowel mucosa.
*+/-Gastric mucosa:
**Foveolar epithelium: champagne flute-like columnar epithelium.
**Oxyntic mucosa: parietal cells (pink) and chief cells (purple).
*+/-Pancreatic epithelium:
**Pancreatic acini.

===Images===

*[http://radiographics.rsna.org/content/24/2/565.long Pancreatic glands and gastric mucosa in a MD (radiographics.rsna.org)].

==See also==
*[[Small intestine]].

==References==
{{Reflist|1}}

[[Category:Diagnosis]]
[[Category:Gastrointestinal pathology]]

Meckel diverticulum

2018-04-08T21:36:30Z

Brigitte: /* Microscopic */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image =
| Width =
| Caption =
| Synonyms =
| Micro = small bowel mucosa, +/-gastric mucosa (foveolar epithelium, oxyntic mucosa), +/-pancreatic epithelium
| Subtypes =
| LMDDx =
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross = small bowel outpouching on antemesenteric aspect ~5 cm long, ~60 cm from the [[ileocecal valve]]
| Grossing =
| Staging =
| Site = [[small intestine]]
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs =
| Symptoms = abdominal pain
| Prevalence = uncommon ~2% of population
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = benign
| Other =
| ClinDDx = [[acute appendicitis]], other causes of abdominal pain
| Tx = surgical removal
}}
'''Meckel diverticulum''' (also '''Meckel's diverticulum'''), is congenital structure of the distal [[small bowel]] that occasionally gets inflamed and may present with [[acute appendicitis]]-like symptoms.

==General==
*Most common congenital anomaly of the gastrointestinal tract.<ref name=pmid15026601>{{Cite journal | last1 = Levy | first1 = AD. | last2 = Hobbs | first2 = CM. | title = From the archives of the AFIP. Meckel diverticulum: radiologic features with pathologic Correlation. | journal = Radiographics | volume = 24 | issue = 2 | pages = 565-87 | month = | year = | doi = 10.1148/rg.242035187 | PMID = 15026601 }}</ref>
**Remnant of the ''omphalomesenteric duct'' - a connection of the yolk sac and midgut.

The rule of 2s:
*2 feet from the terminal ileum
*2% of the population
*2% symptomatic.
*2 inches long.
*2 year old.
*2 types of epithelium - gastric and pancreatic.

Main clinical DDx of a symptomatic Meckel diverticulum:
*[[Appendicitis]].

==Gross==
*Antimesenteric attachement, i.e. a ''Meckel's diverticulum'' hangs off the side opposite of the mesentery.

===Image===
<gallery>
Image:Meckel%27s_Diverticulum_AFIP.jpg | Meckel diverticulum. (AFIP/WC)
</gallery>

==Microscopic==
Features:<ref name=pmid15026601/>
*Small bowel mucosa.
*+/-Gastric mucosa:
**Foveolar epithelium: champagne flute-like columnar epithelium.
**Oxyntic mucosa: parietal cells (pink) and chief cells (purple).
*+/-Pancreatic epithelium:
**Pancreatic acini.

===Images===

*[http://radiographics.rsna.org/content/24/2/565.long Pancreatic glands in a MD (radiographics.rsna.org)].

==See also==
*[[Small intestine]].

==References==
{{Reflist|1}}

[[Category:Diagnosis]]
[[Category:Gastrointestinal pathology]]

Peutz-Jeghers polyp

2018-04-08T21:15:50Z

Brigitte: /* Images */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Peutz-Jeghers_syndrome_polyp.jpg
| Width =
| Caption = Peutz-Jeghers polyp. [[H&E stain]].
| Micro = polyp with branching ''or'' thickened muscularis mucosae, benign epithelium, and lamina propria
| Subtypes =
| LMDDx =
| Stains =
| IHC =
| EM =
| Molecular = STK11 mutation
| IF =
| Gross =
| Grossing =
| Site = [[colon]], [[small bowel]], [[stomach]], others
| Assdx =
| Syndromes = [[Peutz-Jeghers syndrome]]
| Clinicalhx = family history of cancer - esp. gastrointestinal and [[breast cancer|breast]]
| Signs =
| Symptoms =
| Prevalence =
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = benign lesion; suggests increased malignancy risk
| Other =
| ClinDDx = other [[gastrointestinal polyps]]
}}
{{ Infobox external links
| Name = Peutz-Jeghers polyp
| EHVSC = 10180
| pathprotocols =
| wikipedia =
| pathoutlines =
}}
'''Peutz-Jeghers polyp''', abbreviated '''PJP''', is an uncommon [[hamartoma|hamartomous]] [[gastrointestinal polyp]]. It is usually associated with [[Peutz-Jeghers syndrome]].

==General==
===Epidemiology===
Features:<ref name=Ref_PBoD859>{{Ref PBoD|859}}</ref><ref name=pmid12692201>{{Cite journal | last1 = Bronner | first1 = MP. | title = Gastrointestinal inherited polyposis syndromes. | journal = Mod Pathol | volume = 16 | issue = 4 | pages = 359-65 | month = Apr | year = 2003 | doi = 10.1097/01.MP.0000062992.54036.E4 | PMID = 12692201 | url = http://www.nature.com/modpathol/journal/v16/n4/full/3880773a.html }}</ref>
*[[Peutz-Jeghers syndrome]] is autosomal dominant.
*Altered gene: STK11.

===Clinical===
Features:<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/175200 http://www.ncbi.nlm.nih.gov/omim/175200]. Accessed on: 13 July 2010.</ref>
*Melanocytic macules.
**Lips, buccal mucosa, and digits.
**Multiple Peutz-Jeghers polyps.

Increased risk of various neoplasms - primarily:
*Breast and gastrointestinal cancer.<ref name=pmid20581245>{{cite journal |author=Beggs AD, Latchford AR, Vasen HF, ''et al.'' |title=Peutz-Jeghers syndrome: a systematic review and recommendations for management |journal=Gut |volume=59 |issue=7 |pages=975–86 |year=2010 |month=July |pmid=20581245 |doi=10.1136/gut.2009.198499 |url=}}</ref>
*Others tumours:<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/175200 http://www.ncbi.nlm.nih.gov/omim/175200]. Accessed on: 22 December 2010.</ref>
**[[Granulosa cell tumour]].
**[[Sertoli cell tumour]] - esp. with calcification.

==Microscopic==
Features:<ref name=Ref_PBoD859/><ref name=pmid12692201>{{Cite journal | last1 = Bronner | first1 = MP. | title = Gastrointestinal inherited polyposis syndromes. | journal = Mod Pathol | volume = 16 | issue = 4 | pages = 359-65 | month = Apr | year = 2003 | doi = 10.1097/01.MP.0000062992.54036.E4 | PMID = 12692201 | url = http://www.nature.com/modpathol/journal/v16/n4/full/3880773a.html }}</ref>
*Frond-like polyp with all three components of mucosa:
*# Muscosal epithelium (melanotic mucosa, goblet cells).
*# Lamina propria.
*# M. mucosae.

Notes:
*''Frond'' = leaflike expansion.<ref>URL: [http://dictionary.reference.com/browse/frond http://dictionary.reference.com/browse/frond]. Accessed on: 26 July 2011.</ref>
**The '''key''' is "thick" smooth muscle bundles - if one is lucky one sees branching.<ref>C. Streutker. 26 July 2011.</ref>
***"Thick" ~= thickness of muscularis mucosae.

DDx:
*[[Hyperplastic polyp of the stomach]] - should ''not'' have thickened muscle.
**May be confused with PJP as branching may not be apparent.

===Images===
<gallery>
Image:Peutz-Jeghers_syndrome_polyp.jpg | Peutz-Jeghers polyp - intestine (WC/Nephron)
</gallery>
<gallery>
Image:Gastric_Peutz-Jeghers_polyp_-_very_low_mag.jpg | Peutz-Jeghers polyp - stomach - very low mag. (WC/Nephron)
Image: Gastric Peutz-Jeghers polyp - low mag.jpg | Peutz-Jeghers polyp - stomach - low mag. (WC/Nephron)
Image: Gastric Peutz-Jeghers polyp - intermed mag.jpg | Peutz-Jeghers polyp - stomach - intermed. mag. (WC/Nephron)
</gallery>
<gallery>
Image:Colon histology with Peutz-Jeghers polyp.jpg | Peutz-Jeghers (colonic) polyp (WC)
</gallery>

==Sign out==
===Duodenum===
<pre>
POLYPS, DUODENUM, EXCISION:
- PEUTZ-JEGHERS POLYPS (x2) WITH BRUNNER'S GLANDS.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
</pre>

===Colon===
<pre>
POLYP, COLON (40 CM), EXCISION:
- PEUTZ-JEGHERS POLYP.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
</pre>

==See also==
*[[Gastrointestinal tract polyps]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]

Juvenile polyp

2018-04-08T21:11:56Z

Brigitte: /* Images */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Gastric_juvenile_polyp_-_very_low_mag.jpg
| Width =
| Caption = Juvenile polyp (stomach). [[H&E stain]].
| Micro = eroded, smooth or lobulated surface, pedunculated, increased lamina propria (LP) +/- edema,
cystically dilated gland, +/-inflammation
| Subtypes =
| LMDDx = [[inflammatory polyp]], [[hyperplastic polyp of the stomach]], [[Cronkhite-Canada syndrome]]
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = [[gastrointestinal tract polyps]]
| Assdx =
| Syndromes = [[Juvenile polyposis syndrome]]
| Clinicalhx =
| Signs =
| Symptoms =
| Prevalence = uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = benign itself; multiple = marker of increased risk of malignancy
| Other =
| ClinDDx = other [[GI polyps]]
}}
'''Juvenile polyp''', abbreviated '''JP''', is a type of [[hamartoma|hamartomatous]][[Gastrointestinal tract polyps|gastrointestinal polyp]].

In adults, it is known as a '''retention polyp'''.

==General==
*Uncommon.

May be part of a syndrome:
*[[Juvenile polyposis syndrome]] (JPS) - see JPS article for criteria.
*[[Cronkhite-Canada syndrome]].
*[[Cowden syndrome]].

==Gross==
*Mushroom-like shape.

==Microscopic==
Features:<ref name=Ref_PBoD859>{{Ref PBoD|859}}</ref><ref name=pmid12692201>{{Cite journal | last1 = Bronner | first1 = MP. | title = Gastrointestinal inherited polyposis syndromes. | journal = Mod Pathol | volume = 16 | issue = 4 | pages = 359-65 | month = Apr | year = 2003 | doi = 10.1097/01.MP.0000062992.54036.E4 | PMID = 12692201 | url = http://www.nature.com/modpathol/journal/v16/n4/full/3880773a.html }}</ref>
*Eroded, smooth or lobulated surface.
*Pedunculated.
*Increased lamina propria (LP) +/- edema.
*Cystically dilated gland.
*Often inflammed.

Mnemonic ''DIES'' = dilated glands, increased LP & inflammation of the LP, eroded/smooth surface, stalk.

Notes:
*May have nuclear changes like those seen in adenomatous polyps.

DDx:
*[[Inflammatory polyp]].
*[[Hyperplastic polyp of the stomach]] - less lamina propria, foveolar hyperplasia (long tortuous glands).
*[[Cronkhite-Canada syndrome]] - have changes in the surrounding mucosa, clinical findings (nail atrophy, skin pigment, alopecia).

===Images===
<gallery>
Image:Gastric_juvenile_polyp_-_very_low_mag.jpg | Juvenile polyp of the stomach - very low mag. (WC/Nephron)
Image:Gastric_juvenile_polyp_-_2_-_very_low_mag.jpg | Juvenile polyp of the stomach - very low mag. (WC/Nephron)
</gallery>

==IHC==
*Usually none.

Notes:
*IHC can be used if it is suspected to have dysplasia (p53, Ki-67).
**p53 mutations in dysplastic epithelium -- negative stain (normal).

==Sign out==
<pre>
Polyp, Sigmoid Colon, Polypectomy:
- Juvenile polyp/retention polyp.
</pre>

===Block letters===
<pre>
RECTOSIGMOID POLYP, BIOPSY:
- RETENTION POLYP.
</pre>

==See also==
*[[Gastrointestinal tract polyps]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Gastrointestinal pathology]]

Ischemic colitis

2018-04-08T21:06:56Z

Brigitte:

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Ischemic_colitis_-_high_mag.jpg
| Width =
| Caption = Ischemic colitis. [[H&E stain]].
| Micro = withering crypts (colonic epithelium has decreased cytoplasm - NC ratio increased, usually with decreased goblet cells), crypt loss/drop-out, lamina propria hyalinization, submucosa hyalinization, +/-pseudomembranes, +/-vascular thrombi, +/-cryptitis
| Subtypes =
| LMDDx = [[inflammatory bowel disease]], [[radiation colitis]], toxins/drugs, [[infectious colitis]]
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross = dusky appearing, +/-bowel wall thinning, +/-dilation
| Grossing =
| Site = [[colon]]
| Assdx = [[atherosclerosis]]
| Syndromes =
| Clinicalhx = +/-cardiovascular disease
| Signs = +/-blood per rectum, +/-diarrhea (may be non-bloody)
| Symptoms = abdominal pain, tenesmus
| Prevalence =
| Bloodwork = +/-anemia
| Rads =
| Endoscopy =
| Prognosis = poor if severe
| Other =
| ClinDDx = other causes of [[colitis]], esp. [[infectious colitis]]
}}
'''Ischemic colitis''' is inflammation of the [[colon]] due to a compromised blood supply.

It is also known as '''colonic ischemia''' and '''ischemia of the colon'''.
==General==
*May occur together with ''[[ischemic enteritis]]'', in which case it is known as ''ischemic enterocolitis''.

===Etiology===
Anything that leads to vascular occlusion:
*[[Atherosclerosis]].
*[[Vasculitis]].
*Embolization, e.g. thrombotic, foreign body.

Possible associated pathology:
*[[Necrotizing enteritis]] - necrosis of the small bowel only.
*[[Necrotizing enterocolitis]] - necrosis of the small and large bowel.

Closely related:
*[[Radiation colitis]].
*[[Infectious colitis]].

Note:
*Ischemia = compromised blood supply.

===Clinical===
Classic presentation:<ref name=pmid24070152>{{Cite journal | last1 = Tadros | first1 = M. | last2 = Majumder | first2 = S. | last3 = Birk | first3 = JW. | title = A review of ischemic colitis: is our clinical recognition and management adequate? | journal = Expert Rev Gastroenterol Hepatol | volume = 7 | issue = 7 | pages = 605-13 | month = Sep | year = 2013 | doi = 10.1586/17474124.2013.832485 | PMID = 24070152 }}</ref>
*Abdominal pain.
*Urgency to defecate (tenesmus).
*Bloody diarrhea.

Note:
*Diarrhea may be non-bloody.
**This is a poor prognosticator<ref name=pmid20961178>{{Cite journal | last1 = Montoro | first1 = MA. | last2 = Brandt | first2 = LJ. | last3 = Santolaria | first3 = S. | last4 = Gomollon | first4 = F. | last5 = Sánchez Puértolas | first5 = B. | last6 = Vera | first6 = J. | last7 = Bujanda | first7 = L. | last8 = Cosme | first8 = A. | last9 = Cabriada | first9 = JL. | title = Clinical patterns and outcomes of ischaemic colitis: results of the Working Group for the Study of Ischaemic Colitis in Spain (CIE study). | journal = Scand J Gastroenterol | volume = 46 | issue = 2 | pages = 236-46 | month = Feb | year = 2011 | doi = 10.3109/00365521.2010.525794 | PMID = 20961178 }}</ref> and possibly increases the likelihood of not identifying it in time.

==Gross==
Features - location:<ref name=Ref_PBoD852>{{Ref PBoD|852}}</ref>
*Luminal part (mucosa & submucosa) affected - edema.
*Splenic flexture of colon commonly affected (vascular watershed).

Note:
*May have pseudomembranes (classically assoc. with ''C. difficile'' colitis), i.e. mimics an infectious process.
*DDx for pseudomembranes:<ref name=Ref_PBoD837-8>{{Ref PBoD|837-8}}</ref>
**[[C. difficile]] induced pseudomembranous colitis.
**Ischemic colitis.
**Volvulus.
**Necrotizing infections.
**... anything that causes severe mucosal injury.
*Radiologic correlate = bowel wall thickening.

==Microscopic==
Features:
*Withering crypts - '''important'''.
**Colonic epithelium has decreased cytoplasm - [[NC ratio]] increased.
**Usually with decreased goblet cells.
*Crypt loss/drop-out.
**Less intestinal crypts present.
*Lamina propria hyalinization.
**Dense pink material replaces loose connective tissue.
*Submucosa hyalinization.
*+/-[[Cryptitis]].<ref name=pmid11175639>{{Cite journal | last1 = Zhang | first1 = S. | last2 = Ashraf | first2 = M. | last3 = Schinella | first3 = R. | title = Ischemic colitis with atypical reactive changes that mimic dysplasia (pseudodysplasia). | journal = Arch Pathol Lab Med | volume = 125 | issue = 2 | pages = 224-7 | month = Feb | year = 2001 | doi = 10.1043/0003-9985(2001)1250224:ICWARC2.0.CO;2 | PMID = 11175639 }}</ref>
*+/-Pseudomembranes (microscopic):<ref name=Ref_PBoD837-8>{{Ref PBoD|837-8}}</ref>
**Loss of surface epithelium.
**[[PMN]]s in lamina propria.
**+/-Capillary fibrin thrombi.
*+/-Reactive epithelial changes - may mimic dysplasia.<ref name=pmid11175639/>

Notes:
*Pseudomembranes arise from the crypts - considered ''acute''.

DDx:
*[[Inflammatory bowel disease]].
*[[Radiation colitis]].
*Toxins/drugs.
**Rosuvastatin.<ref name=pmid22744258>{{Cite journal | last1 = Tan | first1 = J. | last2 = Pretorius | first2 = CF. | last3 = Flanagan | first3 = PV. | last4 = Pais | first4 = A. | title = Adverse drug reaction: rosuvastatin as a cause for ischaemic colitis in a 64-year-old woman. | journal = BMJ Case Rep | volume = 2012 | issue = | pages = | month = | year = 2012 | doi = 10.1136/bcr.11.2011.5270 | PMID = 22744258 }}</ref>
**[[Cocaine]].<ref name=pmid21237534>{{Cite journal | last1 = Fabra | first1 = I. | last2 = Roig | first2 = JV. | last3 = Sancho | first3 = C. | last4 = Mir-Labrador | first4 = J. | last5 = Sempere | first5 = J. | last6 = García-Ferrer | first6 = L. | title = [Cocaine-induced ischemic colitis in a high-risk patient treated conservatively]. | journal = Gastroenterol Hepatol | volume = 34 | issue = 1 | pages = 20-3 | month = Jan | year = 2011 | doi = 10.1016/j.gastrohep.2010.10.005 | PMID = 21237534 }}</ref>
**[[NSAID]] overdose.<ref name=pmid11736840>{{Cite journal | last1 = Appu | first1 = S. | last2 = Thompson | first2 = G. | title = Gangrenous ischaemic colitis following non-steroidal anti-inflammatory drug overdose. | journal = ANZ J Surg | volume = 71 | issue = 11 | pages = 694-5 | month = Nov | year = 2001 | doi = | PMID = 11736840 }}</ref>
*[[Infectious colitis]].

===Ischemia versus infection on biopsy===
Dignan and Greenson<ref name=pmid9199649>{{Cite journal | last1 = Dignan | first1 = CR. | last2 = Greenson | first2 = JK. | title = Can ischemic colitis be differentiated from C difficile colitis in biopsy specimens? | journal = Am J Surg Pathol | volume = 21 | issue = 6 | pages = 706-10 | month = Jun | year = 1997 | doi = | PMID = 9199649 }}</ref> created a tabular comparison between ischemia and (''C. difficile'') infection.

A modified version of the Dignan-Greenson table (below) shows that the two etiologies generally cannot be separated; however, hyalinization appears to be useful if it is present:
{| class="wikitable sortable"
! Histologic feature
!Ischemia (24 cases)
!Infection (25 cases)
|-
| Atrophic crypts
| 75%
| 24%
|-
| Lamina propria - haemorrhage
| 75%
| 36%
|-
| Lamina propria - hyalizization
| 67%
| 0%
|-
| Diffuse (histologic) pseudomembranes
| 25%
| 4%
|-
| Mucosal necrosis (full thickness)
| 58%
| 28%
|}

===Images===
<gallery>
Image:Ischemic_colitis_-_low_mag.jpg | Ischemic colitis - low mag. (WC/Nephron)
Image:Ischemic_colitis_-_high_mag.jpg | Ischemic colitis - high mag. (WC/Nephron)
Image:Ischemic_colitis_-_very_high_mag.jpg | Ischemic colitis - very high mag. (WC/Nephron)
Image:Colonic_pseudomembranes_low_mag.jpg | Colonic pseudomembranes - low mag. (WC/Nephron)
Image:Colonic_pseudomembranes_intermed_mag.jpg | Colonic pseudomembranes - intermed. mag. (WC/Nephron)
</gallery>
www:
*[http://www.flickr.com/photos/euthman/3385570758/ Ischemic colitis (flickr.com/euthman)].

==Sign out==
===Biopsy===
<pre>
Left Colon, Biopsy:
- Compatible with ischemic colitis (attenuated epithelium, hyalinized
lamina propria, cryptitis).

Comment:
The differential diagnosis includes: drug reaction, infectious etiologies and, less likely, inflammatory bowel disease. Clinical correlation is required.
</pre>

====Block letters====
<pre>
TRANSVERSE COLON, BIOPSY:
- SEVERE ACTIVE COLITIS WITH ATTENUATED EPITHELIAL CYTOPLASM AND ULCERATION.
- CELLULAR DEBRIS.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.

COMMENT:
The attenuated cytoplasm is compatible with ischemia; however, it is not
accompanied with other suggestive findings (crypt drop out, lamina propria
fibrosis, pseudomembranes). The crypt architecture is test tube-like.

The differential diagnosis includes: ischemia, drug reaction, infectious
etiologies and, less likely, inflammatory bowel disease. Clinical
correlation is required.
</pre>

<pre>
COLON, SPLENIC FLEXURE, BIOPSY:
- PATCHY MODERATE ACTIVE COLITIS WITH ATTENUATED EPITHELIAL CYTOPLASM,
FOCALLY DECREASED GOBLET CELLS AND ULCERATION.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.

COMMENT:
The findings are consistent with ischemia; however, they are not diagnostic.

The differential diagnosis includes: ischemia, drug reaction, infectious
etiologies and, less likely, inflammatory bowel disease. Clinical
correlation is required.
</pre>

===Biopsy with nonspecific findings/compatible===
<pre>
Colon, Random Biopsies:
- Mild acute colitis with mild eosinophilia, see comment.
- NEGATIVE for significant architectural distortion.

Comment:
The colitis could be due to ischemia, drug reaction, infection or inflammatory
bowel disease. Clinical correlation is required.
</pre>

===Short version===
<pre>
LEFT COLON AND SIGMOID COLON, RESECTION:
- PSEUDOMEMBRANOUS COLITIS, SEE COMMENT.
- ONE LYMPH NODE NEGATIVE FOR MALIGNANCY ( 0 POSITIVE / 1 ).
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.

COMMENT:
Pseudomembrane formation is a non-specific finding. It is consistent with ischemia;
however, it may be seen in other contexts, including infection. Clinical correlation is
required.
</pre>

===Long version===
<pre>
RECTOSIGMOID, RESECTION:
- BOWEL WALL ISCHEMIA WITH PERFORATION, SEROSITIS, MICROABSCESS FORMATION AND FOCAL
POORLY FORMED PSEUDOMEMBRANES.
- NEGATIVE FOR MALIGNANCY.
- PLEASE SEE COMMENT.

COMMENT:
There is no evidence of inflammatory bowel disease:
The unaffected mucosa does not have obvious architectural distortion. No granulomas are
identified. The inflammation is largely associated with necrosis/ischemic changes
and favoured to be reactive.

The poorly formed pseudomembranes are associated with mural ischemic changes; they do not
specifically suggest an infection in this context.

The blood vessels do not show a vasculitis, or significant atherosclerosis. Thrombi are
seen on several sections and found predominantly in the (smaller) veins.

Considerations are thrombosis, thromboembolism, mechanical vascular compromise, and
infectious etiologies. A vascular compromise is favoured as the underlying cause.

Clinical and radiologic correlation is suggested.
</pre>

===Another long version===
<pre>
SIGMOID COLON, RESECTION:
- BOWEL WALL ISCHEMIA WITH PERFORATION, SEROSITIS, AND FOCAL POORLY FORMED
PSEUDOMEMBRANES.
- MILD ATHEROSCLEROSIS.
- DIVERTICULAR DISEASE.
- TWO LYMPH NODES NEGATIVE FOR MALIGNANCY ( 0 POSITIVE / 2 ).
- PLEASE SEE COMMENT.

COMMENT:
The sections show the changes of acute and chronic ischemic colitis (submucosal fibrosis,
lamina propria hyalinization, focal crypt drop-out, decreased goblet cells, pigmented
macrophages in the lamina propria, intraepithelial neutrophils).

No granulomas are identified. The inflammation is largely associated with
the necrosis/ischemic changes and favoured to be reactive.

The poorly formed pseudomembranes are associated with mural ischemic changes; they do not
specifically suggest an infectious etiology in this context.

The blood vessels do not show a vasculitis. However, focal neutrophilic perivascular
inflammation is seen; this is probably a reactive process. No vascular thrombi are
identified.

The findings are compatible with perforation secondary to a foreign body in the setting of
chronic ischemia.
</pre>

===Micro===
====Negative for ischemic colitis====
The sections show colorectal mucosa with preservation of the crypt density and
epithelium with a normal nuclear-to-cytoplasm ratio. There is no apparent lamina propria
hyalinization. The muscularis mucosa is prominent. Focally, lymphoid aggregates are
present.

No cryptitis is present. Neutrophils are not apparent in the lamina propria. No erosions
are identified.

The epithelium matures appropriately from the crypt base to the surface.

==See also==
*[[Infarction]].
*[[Colon]].
*[[Ischemic enteritis]].
*[[Colonic cast]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Colon]]

Ameloblastoma

2018-04-04T12:53:57Z

Brigitte: /* Images */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Ameloblastoma - high mag.jpg
| Width =
| Caption = Ameloblastoma. [[H&E stain]].
| Synonyms =
| Micro = stellate reticulum (star-shaped cells), tall columnar cells that have palisaded nuclei with reverse polarization, subnuclear vacuolization, +/-giant cells, +/-subepithelial hyalinization (eosinophilic acellular amorphous material)
| Subtypes = solid/multicystic, unicystic
| LMDDx = [[adenomatoid odontogenic tumour]], [[ameloblastic fibroma]]
| Stains =
| IHC =
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = usu. mandible - see ''[[odontogenic tumours and cysts]]''
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs =
| Symptoms =
| Prevalence = uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = locally aggresive, rarely malignant
| Other =
| ClinDDx = [[keratocystic odontogenic tumour]], odontogenic cysts
| Tx =
}}
'''Ameloblastoma''' is an [[odontogenic tumour]].

==General==
*Osteous lesion.
*Usually mandible.<ref>URL: [http://www.waent.org/archives/2010/Vol3-2/20100618-ameloblastoma/jaw-tumor.htm http://www.waent.org/archives/2010/Vol3-2/20100618-ameloblastoma/jaw-tumor.htm]. Accessed on: 30 November 2011.</ref>
**In a review of 3677 cases, the mandible-to-maxilla ratio was 5 to 1.<ref name=pmid7633291>{{Cite journal | last1 = Reichart | first1 = PA. | last2 = Philipsen | first2 = HP. | last3 = Sonner | first3 = S. | title = Ameloblastoma: biological profile of 3677 cases. | journal = Eur J Cancer B Oral Oncol | volume = 31B | issue = 2 | pages = 86-99 | month = Mar | year = 1995 | doi = | PMID = 7633291 }}</ref>
*May arise from an odontogenic cyst,<ref name=pmid10587275>{{Cite journal | last1 = Eversole | first1 = LR. | title = Malignant epithelial odontogenic tumors. | journal = Semin Diagn Pathol | volume = 16 | issue = 4 | pages = 317-24 | month = Nov | year = 1999 | doi = | PMID = 10587275 }}</ref> e.g. [[dentigerous cyst]].<ref name=pmid21957386>{{Cite journal | last1 = Moosvi | first1 = Z. | last2 = Tayaar | first2 = SA. | last3 = Kumar | first3 = GS. | title = Neoplastic potential of odontogenic cysts. | journal = Contemp Clin Dent | volume = 2 | issue = 2 | pages = 106-9 | month = Apr | year = 2011 | doi = 10.4103/0976-237X.83073 | PMID = 21957386 | PMC = 3180832 }}</ref>
*Can be malignant<ref name=pmid15454770>{{Cite journal | last1 = Goldenberg | first1 = D. | last2 = Sciubba | first2 = J. | last3 = Koch | first3 = W. | last4 = Tufano | first4 = RP. | title = Malignant odontogenic tumors: a 22-year experience. | journal = Laryngoscope | volume = 114 | issue = 10 | pages = 1770-4 | month = Oct | year = 2004 | doi = 10.1097/00005537-200410000-00018 | PMID = 15454770 }}</ref> - rare.<ref name=pmid23775022>{{Cite journal | last1 = Chou | first1 = YH. | last2 = Jhuang | first2 = JY. | last3 = Chang | first3 = MH. | last4 = Huang | first4 = WC. | last5 = Hsieh | first5 = MS. | title = Metastasizing Ameloblastoma With Localized Interstitial Spread in the Lung: Report of Two Cases. | journal = Int J Surg Pathol | volume = | issue = | pages = | month = Jun | year = 2013 | doi = 10.1177/1066896913491321 | PMID = 23775022 }}</ref>

===Classification===
Location:
#Intra-osseous.
#*Locally aggressive.
#Peripheral.
#*Benign.

====Subclassification of intra-osseous type====
Histology:
#Solid/multicystic.
#*More commonly reoccur.
#Unicystic.
#*Unlikely to reoccur.
#*Classically found in younger individuals.

==Gross==
*Mass - usu. mandible or maxilla.<ref name=pmid23775022/>
**Mandile most common.

==Microscopic==
Features:<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970616-7 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970616-7]. Accessed on: March 9, 2010.</ref>
*Stellate reticulum - star-shaped cells, found in a developing tooth.
*Tall columnar cells.
**Palisaded nuclei with reverse polarization.
***Reverse polarization of nuclei = nuclei distant from the basement membrane/nuclei at pole opposite of basement membrane.
***Palisaded nuclei = picket fence appearance; columnar-shaped nuclei with long axis perpendicular to the basement membrane -- '''key feature'''.
**Subnuclear vacuolization.
*+/-Giant cells.
*+/-Subepithelial hyalinization (eosinophilic acellular amorphous material).
**Seen deep to the basement membrane.
*Variable morphology (see below - ''morphology'').

DDx (nuclear palisading):
*[[Adenomatoid odontogenic tumour]].
*[[Ameloblastic fibroma]].

===Images===

<gallery>
Image: Ameloblastoma - low mag.jpg | Ameloblastoma - low mag. (WC)
Image: Ameloblastoma - intermed mag.jpg | Ameloblastoma - intermed. mag. (WC)
Image: Ameloblastoma - high mag.jpg | Ameloblastoma - high mag. (WC)
Image: Ameloblastoma - very high mag.jpg | Ameloblastoma - very high mag. (WC)
</gallery>

===Morphology===
*Not prognostic.

Morphologic variants:
*Follicular ameloblastoma (classic appearance).
*Plexiform ameloblastoma (does not have prominent palisading).
*Acanthomatous ameloblastoma.
*Desmoplastic ameloblastoma.
*Basaloid ameloblastoma.

==See also==
*[[Odontogenic tumours and cysts]].

==References==
{{Reflist|2}}

[[Category:Odontogenic tumours and cysts]]
[[Category:Diagnosis]]