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  =
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In general:
In general:
 
*Adrenocortical adenomas are small, circumscribed and have cells with largely bland nuclei and abundant foamy clear or pink cytoplasm.   
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.
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 102: 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]].
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*"Intermediate risk" 200-400 g, no mets, +/-microscopic disease outside adrenal.
*"Intermediate risk" 200-400 g, no mets, +/-microscopic disease outside adrenal.
*"High risk" >400 g, or mets, or gross invasion of adjacent structures.
*"High risk" >400 g, or mets, or gross invasion of adjacent structures.
==DDX==
Diagnostic categories
*Large pink polygonal cell neoplasms.
*Retroperitonial large polygonal cell neoplasms
**Adrenocortical Adenoma
**Pheochromocytoma
**Metastatic Melanoma
**Metastatic Hepatocellular Carcinoma
**Metastatic Renal Cell Carcinoma
**Epithelioid Angiomyolipoma


==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>
*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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