Difference between revisions of "Adrenocortical carcinoma"

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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  =
Line 53: Line 53:


==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===
Line 94: Line 110:
===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]].
Line 122: Line 138:


==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:
Line 135: Line 156:
*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>
*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>
*[[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==

Latest revision as of 23:18, 18 January 2024

Adrenocortical carcinoma
Diagnosis in short

Adrenocortical carcinoma. H&E stain.
Synonyms adrenal cortical carcinoma
LM see below - various criteria, dependent on adult vs pediatric
IHC vimentin +ve, melan A +ve, inhibin-alpha +ve, chromogranin A -ve, EMA -ve, S-100 -ve, SF-1 +ve
Site adrenal gland - cortex
Syndromes Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome
Prevalence uncommon
Radiology adrenal mass, typically large
Prognosis poor
Clin. DDx renal cell carcinoma, other abdominal masses
Treatment surgical excision if feasible

Adrenocortical carcinoma, abbreviated ACC, is a malignant tumour of the adrenal gland cortex.

It is also known as adrenal cortical carcinoma.

General

  • A tumour of both children and adults.
  • 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. 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:

  • Tumour may contain fat.[2]

DDx

Diagnostic categories:

Images

  • ACC - low mag. (WC/Nephron)

  • ACC - intermed. mag. (WC/Nephron)

  • ACC - high mag. (WC/Nephron)

  • ACC with normal adrenal medulla - high mag.

  • Adrenal Cortical Carcinoma - Low power (SKB)

  • Adrenal Cortical Carcinoma - Sheeting (diffuse architecture) - Low power (SKB)

  • Adrenal Cortical Carcinoma - Medium power (SKB)

  • Adrenal Cortical Carcinoma - Medium power (SKB)

  • Adrenal Cortical Carcinoma - Medium power (SKB)

  • Adrenal Cortical Carcinoma - Medium power (SKB)

  • Adrenal Cortical Carcinoma - anaplasia - medium power (SKB)

  • Adrenal Cortical Carcinoma - medium power (SKB)

  • Adrenal Cortical Carcinoma - medium power (SKB)

  • Adrenal Cortical Carcinoma - medium power (SKB)

  • Adrenal Cortical Carcinoma - medium power (SKB)

  • Adrenal Cortical Carcinoma - necrosis - medium power (SKB)

  • Adrenal Cortical Carcinoma - medium power (SKB)

  • Adrenal Cortical Carcinoma - medium power (SKB)

  • Adrenal Cortical Carcinoma, high power (SKB)

  • Adrenal Cortical Carcinoma, high power (SKB)

  • Adrenal Cortical Carcinoma, high power (SKB)

  • Adrenal Cortical Carcinoma - necrosis - high power (SKB)

  • Adrenal Cortical Carcinoma - high power (SKB)

  • Adrenal Cortical Carcinoma - Sheeting (diffuse architecture) - high power (SKB)

  • Adrenal Cortical Carcinoma - high power (SKB)

  • Adrenal Cortical Carcinoma - high power (SKB)

  • Adrenal Cortical Carcinoma - high power (SKB)

www:

Adult

Weiss criteria

Three of the following:[3][4]

  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:[5]

  • 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.[6] and Dehner and Hill.[7]

Dehner and Hill propose a very simple system:[7]

  • "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

  • SF-1 +ve.[8][9]
  • Vimentin +ve.
  • Melan A +ve.
  • Inhibin-alpha +ve.
  • Cytokeratins +ve/-ve.
  • p53 +ve.
  • Ki-67 >5%.
    • Typically 1-2 in adrenal cortical adenomas.[10]

Others:

A panel that may be useful for adenoma versus adrenal cortical carcinoma:[10][14]

  • Beta-catenin, p53, reticulin, inhibin, melan A, Ki-67.

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. ↑ Weiss, LM. (Mar 1984). "Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors.". Am J Surg Pathol 8 (3): 163-9. PMID 6703192.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ 7.0 7.1 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.
  8. ↑ Wang R, Solomon B, Luen SJ, Prall OW, Khoo C, Gill AJ, Lewin J, Sachithanandan N (January 2022). "Pitfalls and progress in adrenocortical carcinoma diagnosis: the utility of a multidisciplinary approach, immunohistochemistry and genomics". Endocrinol Diabetes Metab Case Rep 2022. doi:10.1530/EDM-21-0081. PMC 8789009. PMID 35023475. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8789009/.
  9. ↑ 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 (October 2010). "High diagnostic and prognostic value of steroidogenic factor-1 expression in adrenal tumors". J Clin Endocrinol Metab 95 (10): E161–71. doi:10.1210/jc.2010-0653. PMID 20660055.
  10. ↑ 10.0 10.1 10.2 Arola, J.; Salmenkivi, K.; Liu, J.; Kahri, AI.; Heikkilä, P. (Nov 2000). "p53 and Ki67 in adrenocortical tumors.". Endocr Res 26 (4): 861-5. PMID 11196463.
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ Kovach, AE.; Nucera, C.; Lam, QT.; Nguyen, A.; Dias-Santagata, D.; Sadow, PM.. "Genomic and immunohistochemical analysis in human adrenal cortical neoplasia reveal beta-catenin mutations as potential prognostic biomarker.". Discoveries (Craiova) 3 (2). doi:10.15190/d.2015.32. PMID 26317117.

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