|
|
Line 341: |
Line 341: |
| *[[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:
| |
| <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>
| |
|
| |
|
| ==Neuroblastoma== | | ==Neuroblastoma== |