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.
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===
===Microscopic===
Line 33: 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===
===IHC===
Adrenal cortex:
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.
*Chromogranin A -ve.
*Synaptophysin +ve.
*Synaptophysin +ve.
*Alpha-inhibin +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==
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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 54: Line 93:


==Spironolactone bodies==
==Spironolactone bodies==
===General===
{{Main|Spironolactone bodies}}
Etiology:
*Long-term use of spironolactone.
 
===Microscopic===
Features:<ref name=pmid4131694>{{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>
*Location: zona glomerulosa (where aldosterone is produced).
*Appearance: eosinophilic spherical laminated whorls.
 
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==
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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)].
<gallery>
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==
==Adrenal cytomegaly==
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=Benign neoplasms=
=Benign neoplasms=
==Adrenal cortical adenoma==
===General===
Epidemiology:
*Often an incidental finding.


Pathologic/clinical:  
==Adrenal hemangioma==
*May be hormonally active.
Radiographic incidentalomas but may be large and calcified raising a radiographic ddx of adrenal cortical carcinoma.
*Rare.
*40 and 70 years.
*2:1 female-to-male ratio
<gallery>
Image:Adrenal Hemangioma LP CTR.jpg|Adrenal hemangioma - low power (SKB)
Image:Adrenal Hemangioma MP CTR.jpg|Adrenal hemangioma - medium power (SKB)
</gallery>


*Radiologists are good at identifying adenomas, as they are usually lipid rich and have a characteristic low HU signal.<ref>URL: [http://emedicine.medscape.com/article/376240-overview http://emedicine.medscape.com/article/376240-overview].</ref>
==Adrenal cortical adenoma==
 
{{Main|Adrenal cortical adenoma}}
Indications for excision:<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 }}
</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>
*Lesions >30 mm.
*Hormonally active.
*Non-incidental finding. (???)
 
Notes:
*[[Cushing disease]] is due to the ACTH over-production by the [[pituitary]].
*In cortisol producing tumours (''Cushing syndrome''): atrophy of the non-hyperplastic cortex (due to feedback inhibition from the [[pituitary gland]]).
 
===Microscopic===
Classic features:
*Well-defined cell borders.
*Clear cytoplasm.
*May have foci of [[necrosis]]/degeneration and nuclear atypia.
 
Note:
*In aldosterone producing tumours:
**May extend outside of the capsule (should ''not'' be diagnosed as ''[[adrenal cortical carcinoma]]'').
**No atrophy of non-hyperplastic cortex.
 
DDx:
*Adrenal cortical nodule.<ref name=Ref_EP200>{{Ref EP|200}}</ref>
*[[Adrenal cortical hyperplasia]].
**Hyperplasia is multifocal.<ref>IAV. 18 February 2009.</ref>
*[[Adrenal cortical carcinoma]].


==Pheochromocytoma==
==Pheochromocytoma==
===General===
{{Main|Pheochromocytoma}}
*Considered to be a [[paraganglioma]].<ref name=Ref_EP327>{{Ref EP|327}}</ref>
*Literally means "dusky" (pheo) "colour" (chromo) - dull appearance on gross.
*Tumour arises from adrenal medulla - chromaffin cells.<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
 
Memory device - the rule of 10s:<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
*10% extra-adrenal (e.g. carotid body, organ of Zuckerkandl (neighourhood of aortic bifuration/IMA branch point)).
*10% bilateral.
*10% malignant.
*10% no hypertension.
*25% associated within a syndrome:
*#[[Multiple endocrine neoplasia]] 2A and 2B.
*#[[von Hippel-Lindau syndrome]].
*#[[Neurofibromatosis]] type 1.
*#Familial paraganglioma syndromes - several.
 
====Clinical====
*Classic finding: hypertension.
*Paroxysms (i.e. episodes) of tachycardia, headache, anxiety, hypertension.
 
Laboratory findings (urine):
*Vanillylmandelic acid (VMA).
*Metanephrines.
 
===Microscopic===
Features:<ref>{{Ref PBoD8|1161}}</ref>
*Chief cells:
**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'''.
*Sustentacular cells (structural support cell).
*Often haemorrhagic - highly vascular.
*+/-Nuclear pleomorphism.
 
Notes:
*The nested architecture (Zellballen) is useful for differentiating from [[ACC]].
*[[Metastasis]] sole criteria of malignancy.<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
 
Images:
*[http://commons.wikimedia.org/wiki/File:Carotid_body_tumour_2_low_mag.jpg Carotid body tumour - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Carotid_body_tumour_2_high_mag.jpg Carotid body tumour - high mag. (WC)].
 
DDx:
*[[Adrenal cortical carcinoma]] - ''[[pheochromocytoma versus adrenal cortical carcinoma]]''.
 
=====Pheochromocytoma versus adrenal cortical carcinoma=====
*Pheochromocytoma and adrenal cortical carcinoma overlap histologically.<ref name=pmid20154585>{{Cite journal  | last1 = Sangoi | first1 = AR. | last2 = McKenney | first2 = JK. | title = A tissue microarray-based comparative analysis of novel and traditional immunohistochemical markers in the distinction between adrenal cortical lesions and pheochromocytoma. | journal = Am J Surg Pathol | volume = 34 | issue = 3 | pages = 423-32 | month = Mar | year = 2010 | doi = 10.1097/PAS.0b013e3181cfb506 | PMID = 20154585 }}</ref>
 
Favour pheochromocytoma:
*Small chickenwire-pattern blood vessels, nests, salt-and-pepper chromatin, red blood cell extravasation.
 
Favour adrenal cortical carcinoma:
*Nucleolus, sheeting.
 
====Malignant pheochromoctyoma====
#''Robbins'' says metastases are the sole criteria of malignancy.<ref name=Ref_PCPBoD8_586>{{Ref PCPBoD8|586}}</ref>
#''Thompson'' suggests one can differentiate benign from malignant with the aid of the following:<ref name=Ref_EP259>{{Ref EP|259}}</ref>
#*Marked nuclear atypia.
#*Invasion:
#**Capsular.
#**Vascular.
#*Necrosis.
#*Cellular monotony.
#*Mitoses:
#**Rate.
#**Atypical mitosis.
 
===IHC===
*Chief cells:
**Chromogranin A +ve.
**Synaptophysin +ve.
*Sustentacular cells:
**S100 +ve.
 
===[[Electron microscopy]]===
*Membrane-bound secretory granules.
 
===Sign out===
<pre>
ADRENAL MASS, RIGHT, ADRENALECTOMY:
- PHEOCHROMOCYTOMA.
- SURGICAL MARGIN NEGATIVE FOR PHEOCHROMOCYTOMA.
 
COMMENT:
The tumour cells stains for chromogranin and synaptophysin. S-100 marks the sustentacular cells.
Inhibin is negative in the tumour cells. The immunostaining pattern is consistent with a
pheochromocytoma.
</pre>
 
====Micro====
The sections shows a partially hemorrhagic lesion in the medulla of the adrenal gland that is arranged in nests (Zellballen).  The tumour cells have abundant grey/blue granular cytoplasm, and nuclei with granular chromatin (salt and pepper chromatin). The lesion is surrounded by a compressed rim of adrenal cortex and fibrosis tissue.  The core of the lesion is fibrotic and has clusters of hemosiderin-laden macrophages.
 
There is no capsular invasion. Vascular invasion is not identified.  There is no necrosis. Mitotic activity is not appreciated. 
 
The adrenal cortex is unremarkable.


==Adrenal ganglioneuroma==
==Adrenal ganglioneuroma==
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Images:
Images:
*[http://commons.wikimedia.org/wiki/File:Adrenal_ganglioneuroma_02.JPG Adrenal ganglioneuroma (WC)].
*[http://commons.wikimedia.org/wiki/File:Adrenal_ganglioneuroma_02.JPG Adrenal ganglioneuroma (WC)].
*[http://www.webpathology.com/image.asp?case=84&n=1 Ganglioneuroma (webpathology.com)].
*[http://www.webpathology.com/image.asp?n=2&Case=84 Ganglioneuroma (webpathology.com)].


===Microscopic===
===Microscopic===
Line 295: Line 210:


==Adrenal myelolipoma==
==Adrenal myelolipoma==
:''Myelolipoma'' redirects here.
{{Main|Adrenal myelolipoma}}
===General===
*Benign and rare.
*Typically asymptomatic and hormonally inactive.<ref name=pmid17590837>{{Cite journal  | last1 = Daneshmand | first1 = S. | last2 = Quek | first2 = ML. | title = Adrenal myelolipoma: diagnosis and management. | journal = Urol J | volume = 3 | issue = 2 | pages = 71-4 | month =  | year = 2006 | doi =  | PMID = 17590837 |URL = http://www.urologyjournal.org/index.php/uj/article/view/197/195 }}</ref>
**Symptoms: back or abdominal pain.
*Diagnosis - usu. by abdominal CT.
 
Treatment:
*Watchful waiting if small (<=7 cm) and asymptomatic.<ref name=pmid17590837/>
 
===Microscopic===
Features:<ref name=pmid21927708>{{Cite journal  | last1 = Cha | first1 = JS. | last2 = Shin | first2 = YS. | last3 = Kim | first3 = MK. | last4 = Kim | first4 = HJ. | title = Myelolipomas of both adrenal glands. | journal = Korean J Urol | volume = 52 | issue = 8 | pages = 582-5 | month = Aug | year = 2011 | doi = 10.4111/kju.2011.52.8.582 | PMID = 21927708 | PMC= 3162227 }}</ref>
*Adipose tissue.
*Hematopoietic elements from all three lineages:
*#Erythroid.
*#Myeloid.
*#Megakaryocytic.
*+/-Calcification.<ref name=pmid17590837/>
 
DDx:<ref name=pmid11533079>{{Cite journal  | last1 = Lam | first1 = KY. | last2 = Lo | first2 = CY. | title = Adrenal lipomatous tumours: a 30 year clinicopathological experience at a single institution. | journal = J Clin Pathol | volume = 54 | issue = 9 | pages = 707-12 | month = Sep | year = 2001 | doi =  | PMID = 11533079 | URL = http://jcp.bmj.com/content/54/9/707.full}}</ref>
*[[Angiomyolipoma]] of the kidney.
*[[Lipoma]].
*[[Liposarcoma]].
*[[Teratoma]].
 
Images:
*[http://commons.wikimedia.org/wiki/File:Myelolipoma_histology_HE.jpg Myelolipoma (WC)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3162227/figure/F3/ Myelolipoma (nih.gov)].<ref name=pmid21927708/>
*[http://path.upmc.edu/cases/case165.html Adrenal myelolipoma (upmc.edu)].


==Adenomatoid tumour==
==Adenomatoid tumour==
Line 332: 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:
*[[WC]]:
**[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)].
*www:
**[http://path.upmc.edu/cases/case166.html ACC (upmc.edu)].
 
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 A -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>
*PAX-8 -ve.<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>
*CD10 +ve/-ve -- cannot be used to differentiate from [[RCC]].<ref name=pmid20390424>{{Cite journal  | last1 = Mete | first1 = O. | last2 = Kapran | first2 = Y. | last3 = Güllüoğlu | first3 = MG. | last4 = Kiliçaslan | first4 = I. | last5 = Erbil | first5 = Y. | last6 = Senyürek | first6 = YG. | last7 = Dizdaroğlu | first7 = F. | title = Anti-CD10 (56C6) is expressed variably in adrenocortical tumors and cannot be used to discriminate clear cell renal cell carcinomas. | journal = Virchows Arch | volume = 456 | issue = 5 | pages = 515-21 | month = May | year = 2010 | doi = 10.1007/s00428-010-0901-0 | PMID = 20390424 }}</ref>


==Neuroblastoma==
==Neuroblastoma==
:See also: ''[[olfactory neuroblastoma]]''.
{{Main|Neuroblastoma}}
===General===
Epidemiology:
*Usually paediatric population.
 
Laboratory findings:
*Increased urine homovanillic acid.
 
Predictors of a poor prognosis:<ref name=Ref_PCPBoD8_254>{{Ref PCPBoD8|254}}</ref>
*High mitotic-karyorrhectic index.
*Lack of schwannian stroma.
*>18 months.
*Near ploidy.
*N-MYC amplification.
*Lymph node spread.
*Distant spread.
 
Classification:
*In a grouping known as ''neuroblastic 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).
**[[Ganglioneuroblastoma]] (intermediate).
**Neuroblastoma (aggressive).
 
===Gross===
*Typically an abdominal mass.
**~40% arise in the [[adrenal gland]].<ref name=Ref_PCPBoD8_253>{{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 especially useful for differentiation from lymphoma.
 
DDx:
*[[Small round cell tumours]].
**[[Wilms tumour]].
**Lymphoma.
**[[Hepatoblastoma]].
 
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.
 
===IHC===
*PGP 9.5 +ve.<ref>{{Cite journal  | last1 = Ootsuka | first1 = S. | last2 = Asami | first2 = S. | last3 = Sasaki | first3 = T. | last4 = Yoshida | first4 = Y. | last5 = Nemoto | first5 = N. | last6 = Shichino | first6 = H. | last7 = Chin | first7 = M. | last8 = Mugishima | first8 = H. | last9 = Suzuki | first9 = T. | title = Useful markers for detecting minimal residual disease in cases of neuroblastoma. | journal = Biol Pharm Bull | volume = 31 | issue = 6 | pages = 1071-4 | month = Jun | year = 2008 | doi =  | PMID = 18520032 }}</ref>
**PGP = protein gene product.
*NB-84 +ve.<ref name=pmid9500774>{{Cite journal  | last1 = Miettinen | first1 = M. | last2 = Chatten | first2 = J. | last3 = Paetau | first3 = A. | last4 = Stevenson | first4 = A. | title = Monoclonal antibody NB84 in the differential diagnosis of neuroblastoma and other small round cell tumors. | journal = Am J Surg Pathol | volume = 22 | issue = 3 | pages = 327-32 | month = Mar | year = 1998 | doi =  | PMID = 9500774 }}</ref>
**More sensitive that synaptophysin.
*Synaptophysin +ve.
*CD99 -ve.
 
===EM===
Distinctive EM appearance:<ref name=pmid1196755>{{Cite journal  | last1 = Mackay | first1 = B. | last2 = Masse | first2 = SR. | last3 = King | first3 = OY. | last4 = Butler | first4 = J. | title = Diagnosis of neuroblastoma by electron microscopy of bone marrow aspirates. | journal = Pediatrics | volume = 56 | issue = 6 | pages = 1045-9 | month = Dec | year = 1975 | doi =  | PMID = 1196755 }}</ref>
*Dendritic processes with longitudinally oriented microtubules.
*Membrane bound electron-dense granules (contain catecholamines).
*Desmosomes
**Not seen in [[EWS]], [[RMS]], lymphomas.
*Membrane densities.
 
Pertinent negative:<ref name=pmid1196755/>
*No glycogen.
**Seen in [[EWS]].


=See also=
=See also=
Michael
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