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# | '''Adrenocortical carcinoma''', abbreviated '''ACC''', is a malignant tumour of the [[adrenal gland]] cortex. | ||
It is also known as '''adrenal cortical carcinoma'''. | |||
===General=== | |||
*Prognosis poor, especially 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: | |||
<gallery> | |||
Image:Adrenal_cortical_carcinoma.JPG | ACC - cytology (WC/AFIP) | |||
</gallery> | |||
==Microscopic== | |||
Various criteria exist for this diagnosis. The most widely used is the ''Weiss criteria'', which is a big long clunker. | |||
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> | |||
===Images=== | |||
<gallery> | |||
Image:Adrenal_cortical_carcinoma_-_low_mag.jpg | ACC - low mag. (WC/Nephron) | |||
Image:Adrenal_cortical_carcinoma_-_m_-_high_mag.jpg | ACC - high mag. (WC/Nephron) | |||
Image:Adrenal_cortical_carcinoma_-_high_mag.jpg | ACC with normal adrenal medulla - high mag. (WC/Nephron) | |||
</gallery> | |||
www: | |||
*[http://path.upmc.edu/cases/case166.html ACC (upmc.edu)]. | |||
===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> | |||
==See also== | |||
*[[Adrenal gland]]. | |||
*[[Adrenal cortical adenoma]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Adrenal gland]]. | |||