Adrenocortical carcinoma

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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
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.


  • A tumour of both children and adults.
  • Prognosis poor, especially in adults.



  • +/-Encapsulated.
  • Necrotic-appearing.



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.


  • Tumour may contain fat.[2]


Diagnostic categories:




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.


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.


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


A panel that may be useful for adenoma versus adrenal cortical carcinoma:[8][12]

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

See also


  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. 8.0 8.1 8.2 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.
  9. 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.
  10. 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.
  11. 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.
  12. 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.