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| LMDDx = | | LMDDx = | ||
| Stains = | | Stains = | ||
| IHC = vimentin +ve, melan A +ve, inhibin-alpha +ve, chromogranin A -ve, [[EMA]] -ve, S-100 -ve | | IHC = vimentin +ve, melan A +ve, inhibin-alpha +ve, chromogranin A -ve, [[EMA]] -ve, S-100 -ve, SF-1 +ve | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = | ||
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==General== | ==General== | ||
*A tumour of both children and adults. | |||
*Prognosis poor, especially in adults. | *Prognosis poor, especially in adults. | ||
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==Microscopic== | ==Microscopic== | ||
Various criteria exist for this diagnosis. The most widely used is the ''Weiss criteria'', which is a big long clunker. | Various criteria exist for this diagnosis. The most widely used is the ''Weiss criteria'', which is a big long clunker. This area is prone to regular re-jiggering of criteria and a literature update or expert opinion is recommended prior to signing out one of these rare lesions. | ||
In general: | |||
*Adrenocortical adenomas are small, circumscribed and have cells with largely bland nuclei and abundant foamy clear or pink cytoplasm. | |||
*Adrenocortical carcinomas are large, infiltrative, have fibrous bands and necrosis, and cells with less cytoplasm and more atypia including atypical mitotic figures. | |||
*Adrenocortical adenomas in children; however, can look really ugly. | |||
Notes: | 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> | *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> | ||
====DDx==== | |||
Diagnostic categories: | |||
*[[Large epithelioid tumours|Large pink polygonal cell neoplasms]]. | |||
*Retroperitonial large polygonal cell neoplasms. | |||
**[[Adrenocortical adenoma]]. | |||
**[[Pheochromocytoma]]. | |||
**[[Malignant melanoma|Metastatic melanoma]]. | |||
**[[Hepatocellular carcinoma|Metastatic hepatocellular carcinoma]]. | |||
**[[Renal cell carcinoma|Metastatic renal cell carcinoma]]. | |||
**[[Epithelioid angiomyolipoma]]. | |||
===Images=== | ===Images=== | ||
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===Adult=== | ===Adult=== | ||
====Weiss criteria==== | ====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> | 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><ref name=pmid6703192>{{Cite journal | last1 = Weiss | first1 = LM. | title = Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. | journal = Am J Surg Pathol | volume = 8 | issue = 3 | pages = 163-9 | month = Mar | year = 1984 | doi = | PMID = 6703192 }}</ref> | ||
#High nuclear grade. | #High nuclear grade. | ||
#High mitotic rate; >5/50 HPF (@ 40X obj.) - definition suffers from [[HPFitis]]. | #High mitotic rate; >5/50 HPF (@ 40X obj.) - definition suffers from [[HPFitis]]. | ||
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==IHC== | ==IHC== | ||
*SF-1 +ve.<ref>{{cite journal |authors=Wang R, Solomon B, Luen SJ, Prall OW, Khoo C, Gill AJ, Lewin J, Sachithanandan N |title=Pitfalls and progress in adrenocortical carcinoma diagnosis: the utility of a multidisciplinary approach, immunohistochemistry and genomics |journal=Endocrinol Diabetes Metab Case Rep |volume=2022 |issue= |pages= |date=January 2022 |pmid=35023475 |pmc=8789009 |doi=10.1530/EDM-21-0081 |url=}}</ref><ref>{{cite journal |authors=Sbiera S, Schmull S, Assie G, Voelker HU, Kraus L, Beyer M, Ragazzon B, Beuschlein F, Willenberg HS, Hahner S, Saeger W, Bertherat J, Allolio B, Fassnacht M |title=High diagnostic and prognostic value of steroidogenic factor-1 expression in adrenal tumors |journal=J Clin Endocrinol Metab |volume=95 |issue=10 |pages=E161–71 |date=October 2010 |pmid=20660055 |doi=10.1210/jc.2010-0653 |url=}}</ref> | |||
*Vimentin +ve. | *Vimentin +ve. | ||
*Melan A +ve. | *Melan A +ve. | ||
*Inhibin-alpha +ve. | *Inhibin-alpha +ve. | ||
*Cytokeratins +ve/-ve. | *Cytokeratins +ve/-ve. | ||
*p53 +ve. | |||
**Rarely positive in [[adrenal cortical adenoma]]s.<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> | |||
*Ki-67 >5%. | |||
**Typically 1-2 in adrenal cortical adenomas.<ref name=pmid11196463/> | |||
Others: | Others: | ||
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*S100 -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> | **[[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> | ||
* | *[[PAX8]] -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> | *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> | ||
A panel that may be useful for [[adrenal cortical adenoma|adenoma]] versus adrenal cortical carcinoma:<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><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> | |||
*Beta-catenin, p53, reticulin, inhibin, melan A, Ki-67. | |||
==See also== | ==See also== |
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