Difference between revisions of "Adrenocortical carcinoma"

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#redirect [[Adrenal_gland#Adrenocortical_carcinoma]]
'''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]].

Revision as of 02:25, 23 August 2014

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:

Gross

  • +/-Encapsulated.
  • Necrotic-appearing.

Image:

  • ACC - cytology (WC/AFIP)

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.[2]

Images

  • ACC - low mag. (WC/Nephron)

  • ACC - high mag. (WC/Nephron)

  • ACC with normal adrenal medulla - high mag. (WC/Nephron)

www:

Adult

Weiss criteria

Three of the following:[3]

  1. High nuclear grade.
  2. High mitotic rate; >5/50 HPF (@ 40X obj.) - definition suffers from HPFitis.
  3. Atypical mitoses.
  4. Cleared cytoplasm in >= 25% of tumour cells.
  5. Sheeting (diffuse architecture) in >= 1/3 of tumour cells.
  6. Necrosis in nests.
  7. Venous invasion.
  8. Adrenal sinusoid invasion; lymphovascular space invasion within the adrenal gland.
  9. Capsular invasion.

Volante criteria

There is a simplified set of criteria by Volante et al. - that is not widely used:[4]

  • Reticular network disruption (with reticulin staining).
  • One of the three following:
    1. Abundant mitoses >5/50 high-power fields - definition suffers from HPFitis.
    2. Necrosis.
    3. Vascular invasion.

Pediatric

The criteria in the pediatric setting are somewhat different. This is discussed by Wieneke et al.[5] and Dehner and Hill.[6]

Dehner and Hill propose a very simple system:[6]

  • "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:

See also

References

  1. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1157. ISBN 978-1416031215.
  2. ↑ Heye S, Woestenborghs H, Van Kerkhove F, Oyen R (2005). "Adrenocortical carcinoma with fat inclusion: case report". Abdom Imaging 30 (5): 641–3. doi:10.1007/s00261-004-0281-5. PMID 15688105.
  3. ↑ Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A (2010). "Weiss criteria in large adrenocortical tumors: a validation study". Indian J Pathol Microbiol 53 (2): 222–6. doi:10.4103/0377-4929.64325. PMID 20551521.
  4. ↑ Volante M, Bollito E, Sperone P, et al. (November 2009). "Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification". Histopathology 55 (5): 535–43. doi:10.1111/j.1365-2559.2009.03423.x. PMID 19912359.
  5. ↑ Wieneke JA, Thompson LD, Heffess CS (July 2003). "Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients". Am. J. Surg. Pathol. 27 (7): 867–81. PMID 12826878.
  6. ↑ 6.0 6.1 Dehner LP, Hill DA (2009). "Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?". Pediatr. Dev. Pathol. 12 (4): 284–91. doi:10.2350/08-06-0489.1. PMID 19326954.
  7. ↑ Unger P, Hoffman K, Pertsemlidis D, Thung S, Wolfe D, Kaneko M (May 1991). "S100 protein-positive sustentacular cells in malignant and locally aggressive adrenal pheochromocytomas". Arch. Pathol. Lab. Med. 115 (5): 484–7. PMID 1673596.
  8. ↑ Sangoi, AR.; Fujiwara, M.; West, RB.; Montgomery, KD.; Bonventre, JV.; Higgins, JP.; Rouse, RV.; Gokden, N. et al. (May 2011). "Immunohistochemical distinction of primary adrenal cortical lesions from metastatic clear cell renal cell carcinoma: a study of 248 cases.". Am J Surg Pathol 35 (5): 678-86. doi:10.1097/PAS.0b013e3182152629. PMID 21490444.
  9. ↑ Mete, O.; Kapran, Y.; Güllüoğlu, MG.; Kiliçaslan, I.; Erbil, Y.; Senyürek, YG.; Dizdaroğlu, F. (May 2010). "Anti-CD10 (56C6) is expressed variably in adrenocortical tumors and cannot be used to discriminate clear cell renal cell carcinomas.". Virchows Arch 456 (5): 515-21. doi:10.1007/s00428-010-0901-0. PMID 20390424.

.

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