Difference between revisions of "Adrenal gland"

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[[Image:Gray1183.png|thumb|400px|A drawing of the adrenal glands.]]
'''Adrenal gland''' is a little organ that hangs-out above the [[kidney]].  Pathologists rarely see it.  It uncommonly is affected by tumours.
'''Adrenal gland''' is a little organ that hangs-out above the [[kidney]].  Pathologists rarely see it.  It uncommonly is affected by tumours.


==Anatomy & histology==
==Anatomy & histology==
:''Adrenal cortical rest'' redirects here.
===Anatomy===
===Anatomy===
*Cortex.
*Cortex.
*Medulla.
*Medulla.


===Histology===
Note:
*Adrenal tissue may be associated with gonads or between gonads and adrenal gland proper.<ref>{{Cite journal  | last1 = Barwick | first1 = TD. | last2 = Malhotra | first2 = A. | last3 = Webb | first3 = JA. | last4 = Savage | first4 = MO. | last5 = Reznek | first5 = RH. | title = Embryology of the adrenal glands and its relevance to diagnostic imaging. | journal = Clin Radiol | volume = 60 | issue = 9 | pages = 953-9 | month = Sep | year = 2005 | doi = 10.1016/j.crad.2005.04.006 | PMID = 16124976 }}</ref>
 
===Microscopic===
It is composed of a ''cortex'' and a ''medulla''.
It is composed of a ''cortex'' and a ''medulla''.


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#*Marked eosinophilia of cytoplasm - '''key feature'''.
#*Marked eosinophilia of cytoplasm - '''key feature'''.
#*Granular/reticular cytoplasm.
#*Granular/reticular cytoplasm.
Note:
*Normal cortex may not be completely encapsulated, i.e. the adrenal capsule may have defects.<ref>{{Ref_H4P4|1236}}</ref>
**In other words: the cortex may "spill" into the surrounding fat.


====Medulla====
====Medulla====
Line 29: Line 38:


Produce ''NED'': norepinephrine, epinephrine, dopamine.
Produce ''NED'': norepinephrine, epinephrine, dopamine.
=====Images=====
<gallery>
Image:Adrenal_gland_(medulla).JPG | Adrenal medulla. (WC)
Image:Adrenal_cortical_carcinoma_-_low_mag.jpg | Adrenal cortex & medulla (right of image), and tumour (left of image). (WC/Nephron)
</gallery>
<gallery>
Image:Adrenal rest - epididymis -- low mag.jpg | Adrenal rest - low mag. (WC/Nephron)
Image:Adrenal rest - epididymis -- intermed mag.jpg | Adrenal rest - intermed. mag. (WC/Nephron)
Image:Adrenal rest - epididymis -- high mag.jpg | Adrenal rest - high mag. (WC/Nephron)
</gallery>
=====www=====
*[http://www.webpathology.com/image.asp?case=78&n=5 Adrenal medulla (webpathology.com)].
===IHC===
Adrenal cortex:<ref name=pmid18579979>{{Cite journal  | last1 = De Padua | first1 = M. | last2 = Rajagopal | first2 = V. | title = Myxoid adrenal adenoma with focal pseudoglandular pattern. | journal = Indian J Med Sci | volume = 62 | issue = 5 | pages = 199-203 | month = May | year = 2008 | doi =  | PMID = 18579979 }}</ref>
*Chromogranin A -ve.
*Synaptophysin +ve.
*Alpha-inhibin +ve.
*Vimentin +ve.
*Melan A +ve.
*AE1/AE3 -ve.
*RCC -ve (0 +ve of 63 cases<ref name=pmid21490444>{{Cite journal  | last1 = Sangoi | first1 = AR. | last2 = Fujiwara | first2 = M. | last3 = West | first3 = RB. | last4 = Montgomery | first4 = KD. | last5 = Bonventre | first5 = JV. | last6 = Higgins | first6 = JP. | last7 = Rouse | first7 = RV. | last8 = Gokden | first8 = N. | last9 = McKenney | first9 = JK. | title = Immunohistochemical distinction of primary adrenal cortical lesions from metastatic clear cell renal cell carcinoma: a study of 248 cases. | journal = Am J Surg Pathol | volume = 35 | issue = 5 | pages = 678-86 | month = May | year = 2011 | doi = 10.1097/PAS.0b013e3182152629 | PMID = 21490444 }}</ref>).
*EMA -ve (0 +ve of 63 cases<ref name=pmid21490444/>).
A panel that may be useful for [[adrenal cortical adenoma|adenoma]] versus [[adrenal cortical carcinoma|carcinoma]]:<ref name=pmid26317117>{{Cite journal  | last1 = Kovach | first1 = AE. | last2 = Nucera | first2 = C. | last3 = Lam | first3 = QT. | last4 = Nguyen | first4 = A. | last5 = Dias-Santagata | first5 = D. | last6 = Sadow | first6 = PM. | title = Genomic and immunohistochemical analysis in human adrenal cortical neoplasia reveal beta-catenin mutations as potential prognostic biomarker. | journal = Discoveries (Craiova) | volume = 3 | issue = 2 | pages =  | month =  | year =  | doi = 10.15190/d.2015.32 | PMID = 26317117 }}
</ref><ref name=pmid11196463>{{Cite journal  | last1 = Arola | first1 = J. | last2 = Salmenkivi | first2 = K. | last3 = Liu | first3 = J. | last4 = Kahri | first4 = AI. | last5 = Heikkilä | first5 = P. | title = p53 and Ki67 in adrenocortical tumors. | journal = Endocr Res | volume = 26 | issue = 4 | pages = 861-5 | month = Nov | year = 2000 | doi =  | PMID = 11196463 }}</ref>
*Beta-catenin, p53, reticulin, inhibin, melan A, Ki-67.


==Clinical==
==Clinical==
Patients getting a bilat. adrenalectomy get pre-treatment with steroids.<ref>URL:
Patients getting a bilateral adrenalectomy get pre-treatment with steroids.<ref>URL:
[http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART]. Accessed on: 21 August 2010.</ref>
[http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART http://www3.interscience.wiley.com/cgi-bin/fulltext/119909358/PDFSTART]. Accessed on: 21 August 2010.</ref>


Adrenal insuff. may be immediately post-op.<ref>URL: [http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516 http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516]. Accessed on: 21 August 2010.</ref>
Adrenal insufficiency is an immediate danger post-op.<ref>URL: [http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516 http://ats.ctsnetjournals.org/cgi/content/full/62/5/1516]. Accessed on: 21 August 2010.</ref>


=Benign=
=Benign=
The section covers non-neoplastic pathologies of the adrenal gland. These uncommonly come to the pathologist.
The section covers non-neoplastic pathologies of the adrenal gland. These uncommonly come to the pathologist.


*Adrenal incidentalomas<ref>{{Cite journal  | last1 = Aljabri | first1 = KS. | last2 = Bokhari | first2 = SA. | last3 = Alkeraithi | first3 = M. | title = Adrenal hemangioma in a 19-year-old female. | journal = Ann Saudi Med | volume = 31 | issue = 4 | pages = 421-3 | month =  | year =  | doi = 10.4103/0256-4947.76411 | PMID = 21293064 }}</ref>
**Adrenal tumors
**Greater than 1 cm
**Identified on imaging performed for other indications
*Found in up to 10% of patients undergoing abdominal imaging.
*Management problematic
** Guidelines incorporate lesion size, functional status and imaging features.
**Resection is generally advocated for
***Functioning lesions.
***Radiographic features suggestive of malignancy.
***Growth during observation.
==Stress response==
==Stress response==
*In fetuses - fat content increases due to stress<ref name=pmid964978>{{cite journal |author=Becker MJ, Becker AE |title=Fat distribution in the adrenal cortex as an indication of the mode of intrauterine death |journal=Hum. Pathol. |volume=7 |issue=5 |pages=495–504 |year=1976 |month=September |pmid=964978 |doi= |url=}}</ref> -- see: ''[[Fetal_autopsy#Adrenal_fetal_fat_pattern]]''.
*In fetuses - fat content increases due to stress<ref name=pmid964978>{{cite journal |author=Becker MJ, Becker AE |title=Fat distribution in the adrenal cortex as an indication of the mode of intrauterine death |journal=Hum. Pathol. |volume=7 |issue=5 |pages=495–504 |year=1976 |month=September |pmid=964978 |doi= |url=}}</ref> -- see: ''[[Fetal_autopsy#Adrenal_fetal_fat_pattern]]''.
Line 44: Line 93:


==Spironolactone bodies==
==Spironolactone bodies==
Features:<ref>{{cite journal |author=Kovacs K, Horvath E, Singer W |title=Fine structure and morphogenesis of spironolactone bodies in the zona glomerulosa of the human adrenal cortex |journal=J. Clin. Pathol. |volume=26 |issue=12 |pages=949-57 |year=1973 |month=December |pmid=4131694 |pmc=477936 |doi= |url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=4131694}}</ref>
{{Main|Spironolactone bodies}}
*Location: zona glomerulosa (where aldosterone is produced).
*Appearance: eosinophilic spherical laminated whorls.
*Etiology: long-term use of spironolactone.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Spironolactone_bodies_high_mag.jpg SB - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Spironolactone_bodies.png SB (circled) - cropped high mag. (WC)].


==Hemorrhagic adrenalitis==
==Hemorrhagic adrenalitis==
*[[AKA]] ''Waterhouse-Friderichsen syndrome''.
===General===
===General===
*[[AKA]] ''Waterhouse-Friderichsen syndrome''.
*Classically thought to be only due to ''Neisseria meningitidis''; however, more recently also associated with ''Staphylococcus aureus'',<ref name=pmid16177250>{{cite journal |author=Adem PV, Montgomery CP, Husain AN, ''et al.'' |title=Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children |journal=N. Engl. J. Med. |volume=353 |issue=12 |pages=1245–51 |year=2005 |month=September |pmid=16177250 |doi=10.1056/NEJMoa044194 |url=}}</ref> and ''Streptococcus pneumoniae''.<ref name=pmid14747454>{{cite journal |author=Hamilton D, Harris MD, Foweraker J, Gresham GA |title=Waterhouse-Friderichsen syndrome as a result of non-meningococcal infection |journal=J. Clin. Pathol. |volume=57 |issue=2 |pages=208–9 |year=2004 |month=February |pmid=14747454 |pmc=1770213 |doi= |url=}}</ref>
*Classically thought to be only due to ''Neisseria meningitidis''; however, more recently also associated with ''Streptococcus aureus''.<ref name=pmid16177250>{{cite journal |author=Adem PV, Montgomery CP, Husain AN, ''et al.'' |title=Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children |journal=N. Engl. J. Med. |volume=353 |issue=12 |pages=1245–51 |year=2005 |month=September |pmid=16177250 |doi=10.1056/NEJMoa044194 |url=}}</ref><ref name=pmid14747454>{{cite journal |author=Hamilton D, Harris MD, Foweraker J, Gresham GA |title=Waterhouse-Friderichsen syndrome as a result of non-meningococcal infection |journal=J. Clin. Pathol. |volume=57 |issue=2 |pages=208–9 |year=2004 |month=February |pmid=14747454 |pmc=1770213 |doi= |url=}}</ref>


===Gross===
===Gross===
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Image: [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770213/figure/f1/ Haemorrhage in adrenal (nih.gov)].
Image: [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770213/figure/f1/ Haemorrhage in adrenal (nih.gov)].


==Adrenocortical insufficency disease==
<gallery>
*[[AKA]] Addison's disease.
Image:Adrenal WaterhouseFriderichse EColiSepsis LP PA.JPG|Adrenal hemorrhage -low power - E. coli sepsis (SKB)
Image:Adrenal WaterhouseFriderichse EColiSepsis MP PA.JPG|thumb|Adrenal hemorrhage - medium power - E. coli sepsis (SKB)
</gallery>
 
==Adrenal cytomegaly==
*[[AKA]] ''adrenocortical cytomegaly''.
*[[AKA]] ''adrenal gland with cytomegaly''.
 
===General===
===General===
*Adrenal insufficiency.
May be associated with:<ref>URL: [http://www.humpath.com/?adrenal-cytomegaly http://www.humpath.com/?adrenal-cytomegaly]. Accessed on: 3 January 2012.</ref>
*[[Beckwith-Wiedemann syndrome]].
* Prematurity.
* Rh-incompatibility.<ref name=pmid4336262>{{Cite journal  | last1 = Aterman | first1 = K. | last2 = Kerenyi | first2 = N. | last3 = Lee | first3 = M. | title = Adrenal cytomegaly. | journal = Virchows Arch A Pathol Pathol Anat | volume = 355 | issue = 2 | pages = 105-22 | month =  | year = 1972 | doi =  | PMID = 4336262 |URL = http://www.springerlink.com/content/v06w5250415jr818/ }}</ref>
 
===Microscopic===
Features:
*Large cells in the adrenal cortex.<ref name=pmid4336262/>
 
==Addison disease==
===General===
*Chronic adrenocortical insufficiency.


Clinical:
Clinical:
Line 84: Line 144:
DDx:<ref name=Ref_PBoD8_1155>{{Ref PBoD8|1155}}</ref>
DDx:<ref name=Ref_PBoD8_1155>{{Ref PBoD8|1155}}</ref>
*Autoimmune.
*Autoimmune.
*Tuberculosis.
*[[Tuberculosis]].
*[[HIV|AIDS]].
*[[HIV|AIDS]].
*Malignancy.
*Malignancy.
Line 101: Line 161:


=Benign neoplasms=
=Benign neoplasms=
==Adenomas==
Radiology<ref>URL: [http://emedicine.medscape.com/article/376240-overview http://emedicine.medscape.com/article/376240-overview].</ref>
*Radiologists are good at identifying adenomas, as they are usually lipid rich and have a characteristic low HU signal.


Treatment is excision if...<ref name=pmid10870039>{{Cite journal  | last1 = Luton | first1 = JP. | last2 = Martinez | first2 = M. | last3 = Coste | first3 = J. | last4 = Bertherat | first4 = J. | title = Outcome in patients with adrenal incidentaloma selected for surgery: an analysis of 88 cases investigated in a single clinical center. | journal = Eur J Endocrinol | volume = 143 | issue = 1 | pages = 111-7 | month = Jul | year = 2000 | doi =  | PMID = 10870039 }}
==Adrenal hemangioma==
</ref><ref name=pmid19035218>{{Cite journal  | last1 = Liu | first1 = XK. | last2 = Liu | first2 = XJ. | last3 = Dong | first3 = X. | last4 = Kong | first4 = CZ. | title = [Clinical research about treatment for adrenal incidentalomas] | journal = Zhonghua Wai Ke Za Zhi | volume = 46 | issue = 11 | pages = 832-4 | month = Jun | year = 2008 | doi =  | PMID = 19035218 }}</ref>
Radiographic incidentalomas but may be large and calcified raising a radiographic ddx of adrenal cortical carcinoma.
*Lesions >30 mm.
*Rare.
*Hormonally active.
*40 and 70 years.
*Non-incidental finding. (???)
*2:1 female-to-male ratio
 
<gallery>
===Hyperplasia vs. adenoma===
Image:Adrenal Hemangioma LP CTR.jpg|Adrenal hemangioma - low power (SKB)
*Hyperplasia is multifocal.<ref>IAV. 18 February 2009.</ref>
Image:Adrenal Hemangioma MP CTR.jpg|Adrenal hemangioma - medium power (SKB)
</gallery>


==Adrenal cortical adenoma==
==Adrenal cortical adenoma==
===General===
{{Main|Adrenal cortical adenoma}}
Epidemiology:
*Often an incidental finding.


Pathologic/clinical:
==Pheochromocytoma==
*May be hormonally active.
{{Main|Pheochromocytoma}}
 
===Microscopic===
Classic features:
*Well-defined cell borders.
*Clear cytoplasm.
*May have foci of [[necrosis]]/degeneration and nuclear atypia.


In aldosterone producing tumours:
==Adrenal ganglioneuroma==
*May extend outside of the capsule (should ''not'' be diagnosed as ''adrenal cortical carcinoma'').
{{Main|Ganglioneuroma}}
*No atrophy of non-hyperplastic cortex.


In cortisol producing tumours (''Cushing syndrome''):
*Atrophy of the non-hyperplastic cortex (due to feedback inhibition from the [[pituitary gland]]).
Notes:
*[[Cushing disease]] is due to the ACTH over-production by the [[pituitary]].
==Pheochromocytoma==
===General===
===General===
*Considered to be a [[paraganglioma]].<ref name=Ref_EP327>{{Ref EP|327}}</ref>
*May be retroperitoneal.
*Literally means "dusky" (pheo) "colour" (chromo) - dull appearance on gross.
*Multiple ganglioneuromas may be due to [[multiple endocrine neoplasia IIb]].
*Tumour arises from medulla


Clinical:
===Gross===
*Classic finding: hypertension.
*Solid.
*Paroxysms (i.e. episodes) of tachycardia, headache, anxiety, hypertension.
*White.
*Firm.
*Well-circumscribed.
*May be nodular.


Laboratory findings (urine):
DDx (gross):
*Vanillylmandelic acid (VMA).
*[[Leiomyoma]].
*Metanephrines.


Epidemiology:
Images:
*May be associated with [[multiple endocrine neoplasia]] 2a and 2b.
*[http://commons.wikimedia.org/wiki/File:Adrenal_ganglioneuroma_02.JPG Adrenal ganglioneuroma (WC)].
*[http://www.webpathology.com/image.asp?n=2&Case=84 Ganglioneuroma (webpathology.com)].


===Microscopic===
Features:<ref>{{Ref PBoD8|1161}}</ref>
*Usu. polygonal cells, may be spindled.
*Arranged in cell nests - "Zellballen" (literally ''cell balls'') - '''key feature'''.
*Stippled chromatin ([[AKA]] salt and pepper chromatin) - coarsely granular chromatin.
*Granular cytoplasm, often basophilic - '''important'''.
*Often haemorrhagic - highly vascular.
*+/-Nuclear pleomorphism.
Notes:
*The nested architecture (Zellballen) is useful for differentiating from [[ACC]].
===IHC===
*Chromogranin +ve.
*Synaptophysin +ve.
*S100 +ve (sustentacular cells).
===[[EM]]===
*Membrane-bound secretory granules.
==Ganglioneuroma==
===Microscopic===
===Microscopic===
Features:
Features:
Line 186: Line 209:
*[http://commons.wikimedia.org/wiki/File:Ganglion_high_mag.jpg Ganglion - benign (WC)].
*[http://commons.wikimedia.org/wiki/File:Ganglion_high_mag.jpg Ganglion - benign (WC)].


See: ''[[CNS tumours]]''.
==Adrenal myelolipoma==
 
{{Main|Adrenal myelolipoma}}
==Myelolipoma==


==Adenomatoid tumour==
==Adenomatoid tumour==
Line 197: Line 219:
*[[AKA]] ''adrenal cortical carcinoma''.
*[[AKA]] ''adrenal cortical carcinoma''.
*Abbreviated ''ACC''.
*Abbreviated ''ACC''.
 
{{Main|Adrenocortical carcinoma}}
===General===
*Prognosis sucks, esp. in adults.
 
Epidemiology:
*May be associated with a syndrome:<ref name=Ref_PBoD8_1157>{{Ref PBoD8|1157}}</ref>
**[[Li-Fraumeni syndrome]].
**[[Beckwith-Wiedemann syndrome]].
 
===Gross===
*+/-Encapsulated.
*Necrotic-appearing.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Adrenal_cortical_carcinoma.JPG ACC - gross (WC)].
 
===Microscopic===
Various criteria exist for this diagnosis.  The most widely used is the ''Weiss criteria'', which is a big long clunker.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Adrenal_cortical_carcinoma_-_low_mag.jpg ACC - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Adrenal_cortical_carcinoma_-_m_-_high_mag.jpg ACC - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Adrenal_cortical_carcinoma_-_high_mag.jpg ACC with normal adrenal medulla - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Adrnal_cortical_carcinoma.JPG ACC - cytology (AFIP/WC)].
 
Notes:
*Tumour may contain fat.<ref name=pmid15688105>{{cite journal |author=Heye S, Woestenborghs H, Van Kerkhove F, Oyen R |title=Adrenocortical carcinoma with fat inclusion: case report |journal=Abdom Imaging |volume=30 |issue=5 |pages=641–3 |year=2005 |pmid=15688105 |doi=10.1007/s00261-004-0281-5 |url=}}</ref>
 
====Adult====
=====Weiss criteria=====
Three of the following:<ref name=pmid20551521>{{cite journal |author=Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A |title=Weiss criteria in large adrenocortical tumors: a validation study |journal=Indian J Pathol Microbiol |volume=53 |issue=2 |pages=222–6 |year=2010 |pmid=20551521 |doi=10.4103/0377-4929.64325 |url=}}</ref>
#High nuclear grade.
#High mitotic rate; >5/50 HPF (@ 40X obj.) - definition suffers from [[HPFitis]].
#Atypical mitoses.
#Cleared cytoplasm in >= 25% of tumour cells.
#Sheeting (diffuse architecture) in >= 1/3 of tumour cells.
#Necrosis in nests.
#Venous invasion.
#Adrenal sinusoid invasion; lymphovascular space invasion within the adrenal gland.
#Capsular invasion.
 
=====Volante criteria=====
There is a simplified set of criteria by ''Volante et al.'' - that is not widely used:<ref name=pmid19912359>{{cite journal |author=Volante M, Bollito E, Sperone P, ''et al.'' |title=Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification |journal=Histopathology |volume=55 |issue=5 |pages=535–43 |year=2009 |month=November |pmid=19912359 |doi=10.1111/j.1365-2559.2009.03423.x |url=}}</ref>
*Reticular network disruption (with reticulin staining).
*One of the three following:
*#Abundant mitoses >5/50 high-power fields - definition suffers from [[HPFitis]].
*#Necrosis.
*#Vascular invasion.
 
====Pediatric====
The criteria in the pediatric setting are somewhat different.  This is discussed by Wieneke ''et al.''<ref name=pmid12826878>{{cite journal |author=Wieneke JA, Thompson LD, Heffess CS |title=Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients |journal=Am. J. Surg. Pathol. |volume=27 |issue=7 |pages=867–81 |year=2003 |month=July |pmid=12826878 |doi= |url=}}</ref> and Dehner and Hill.<ref name=pmid19326954>{{cite journal |author=Dehner LP, Hill DA |title=Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors? |journal=Pediatr. Dev. Pathol. |volume=12 |issue=4 |pages=284–91 |year=2009 |pmid=19326954 |doi=10.2350/08-06-0489.1 |url=}}</ref>
 
Dehner and Hill propose a very simple system:<ref name=pmid19326954/>
*"Low risk" < 200 g & confined to the adrenal.
*"Intermediate risk" 200-400 g, no mets, +/-microscopic disease outside adrenal.
*"High risk" >400 g, or mets, or gross invasion of adjacent structures.
 
===IHC===
*Vimentin +ve.
*Melan A +ve.
*Inhibin-alpha +ve.
*Cytokeratins +ve/-ve.
 
Others:
*Synaptophysin +ve/-ve.
*Chromogranin -ve.
**Pheochromocytoma +ve.
*EMA -ve.
**[[Renal cell carcinoma]] +ve.
*S100 -ve.
**Pheochromocytoma +ve (sustentacular cells).<ref>{{cite journal |author=Unger P, Hoffman K, Pertsemlidis D, Thung S, Wolfe D, Kaneko M |title=S100 protein-positive sustentacular cells in malignant and locally aggressive adrenal pheochromocytomas |journal=Arch. Pathol. Lab. Med. |volume=115 |issue=5 |pages=484–7 |year=1991 |month=May |pmid=1673596 |doi= |url=}}</ref>
 
==Malignant pheochromoctyoma==
*Like the description in ''benign neoplasms''.
*Differentiated from benign pheochromocytoma by mets - often aided by radiologic report.
*Features useful for differentiating benign from malignant:<ref name=Ref_EP259>{{Ref EP|259}}</ref>
**Marked nuclear atypia.
**Invasion:
***Capsular.
***Vascular.
**Necrosis.
**Cellular monotony.
**Mitoses:
***Rate.
***Atypical mitosis.


==Neuroblastoma==
==Neuroblastoma==
:See also: ''[[olfactory neuroblastoma]]''.
{{Main|Neuroblastoma}}
===General===
Laboratory:
*Increased urine homovanillic acid.
 
Epidemiology:
*Usually paediatric population.
 
Classification:
*In a grouping known as ''neuoblastic tumours'' which includes:<ref name=pmid10421272>{{cite journal |author=Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B |title=Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee |journal=Cancer |volume=86 |issue=2 |pages=349–63 |year=1999 |month=July |pmid=10421272 |doi= |url=}}</ref>
**[[Ganglioneuroma]] (benign).
**Ganglioblastoma (intermediate).
**Neuroblastoma (aggressive).
===Gross===
*~40% of in the [[adrenal gland]].<ref>{{Ref PCPBoD8|253}}</ref>
 
===Microscopic===
Features:<ref name=pmid18635637>{{cite journal |author=Chung EM, Murphey MD, Specht CS, Cube R, Smirniotopoulos JG |title=From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit |journal=Radiographics |volume=28 |issue=4 |pages=1193–214 |year=2008 |pmid=18635637 |doi=10.1148/rg.284085013 |url=}}</ref>
*[[small round cell tumour|Small round blue cell]]s separated by thin (pink) fibrous septa.
*Homer-Wright rosettes.
**Rosette with a small (~100 micrometers - diameter) meshwork of fibers (neuropil) at the centre.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
*Neuropil-like stroma = paucicellular stroma with a cotton candy-like appearance; see comparison below.
**>50% neuropil-like stroma -- otherwise it's a [[ganglioneurona]] or ganglioblastoma.
 
Notes:
*The fibrous septa are esp. useful for differentiation from lymphoma.
 
DDx:
*[[Small round cell tumours]].
 
Images:
*[http://radiographics.rsna.org/content/28/4/1193/F42.expansion Neuroblastoma (radiographics.rsna.org)].<ref>URL: [http://radiographics.rsna.org/content/28/4/1193.full http://radiographics.rsna.org/content/28/4/1193.full]. Accessed on: 12 January 2011.</ref>
*[http://commons.wikimedia.org/wiki/File:Neuroblastoma_rosettes.jpg Neuroblastoma (WC)].
*[http://farm3.static.flickr.com/2259/2274260465_afbea05f9b.jpg Neuroblastoma (flickr.com)].
 
====Schwannian vs. neuropil====
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
| Feature
| Schwannian
| Neuropil
|-
| Cellularity
| high ~ spacing of cells < 30 µm
| low ~ spacing of cells > 100 µm
|-
| Fibrillary
| yes, long fine strands
| no
|-
| Associations
| ganglion cells
| neuroblasts
|-
| Cytoplasmic vacuolation
| yes
| ?
|-
|}
 
====Classification/grading====
Commonly grouped by the ''Shimada classification'', which depends on the presence a number of things including:
*Mitoses/karyorrhectic cells.
*Molecular abnormalities.


=See also=
=See also=
Michael
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