Adrenocortical carcinoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Ahmad Al Maradni, M.D. [3] Mohammed Abdelwahed M.D[4]


Adrenocortical carcinoma can be classified according to hormone production, and histological appearance. ACC may secrete cortisol, aldosterone, testosterone or estrogen. Other variants include oncocytic adrenal cortical carcinoma, myxoid adrenal cortical carcinoma, and carcinosarcoma. According to the TNM staging system, there are four stages of adrenocortical cancer based on the tumor size, lymph nodes, and distant metastasis. Each stage is assigned a number and letter that designates the number of lymph nodes involved and presence/absence of distant metastasis.


Adrenocortical carcinomas are classified based on:[1]

Hormones production:


A Functional adrenocortical carcinoma may produce one or more of the following hormones:[2]


A nonfunctional adrenocortical carcinoma does not procuce any of the above hormones.


Adrenocortical carcinoma can be classified according to the differentiation of the tissue under the microscope into:

  • Well-differentiated
  • Intermediate differentiated
  • Poorly differentiated
  • Anaplastic


  • Oncocytic adrenal cortical carcinoma
  • Myxoid adrenal cortical carcinoma
  • Carcinosarcoma
  • Adenosquamous adrenocortical carcinoma
  • Clear cell adrenal cortical carcinoma

Staging of adrenocortical carcinoma

The AJCC has designated staging by TNM to define adrenocortical carcinoma: [3]

Adrenal cancer TNM staging
Stage Description
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
T1 Tumor ≤5 cm in greatest dimension limited to the adrenals
T2 Tumor >5 cm in greatest dimension, limited to the adrenals
T3 Tumor of any size with local invasion, but not invading adjacent organs
T4 Tumor of any size with invasion of adjacent organs
Regional Lymph Nodes (N)
Stage Description
NX Regional lymph node cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
Distant Metastasis (M)
Stage Description
M0 No distant metastasis
M1 Distant metastasis
Anatomic Stage/Prognostic Groups
Stage T N M
I T1 N0 M0
II T2 N0 M0
III T1 N1 M0
T2 N1 M0
T3 N0 M0
IV T3 N1 M0
T4 N0 M0
T4 N1 M0
Any T Any N M1

A new study showed staging system that incorporates the patient's age better and predicts 5-year survival among patients with stages I/II ACC. Consideration should be given to include age in staging for ACC, because it may better inform providers about treatment and prognosis.[4]


  1. de Krijger RR, Papathomas TG (2012). "Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants". Virchows Arch. 460 (1): 9–18. doi:10.1007/s00428-011-1166-y. PMC 3267029. PMID 22086150.
  2. Ganeshan D, Bhosale P, Kundra V (2012). "Current update on cytogenetics, taxonomy, diagnosis, and management of adrenocortical carcinoma: what radiologists should know". AJR Am J Roentgenol. 199 (6): 1283–93. doi:10.2214/AJR.11.8282. PMID 23169720.
  3. Edge SB, Compton CC (2010). "The American Joint Committee on Cancer: the 7th edition of the AJCC cancer staging manual and the future of TNM". Ann Surg Oncol. 17 (6): 1471–4. doi:10.1245/s10434-010-0985-4. PMID 20180029.
  4. Asare EA, Wang TS, Winchester DP, Mallin K, Kebebew E, Sturgeon C (2014). "A novel staging system for adrenocortical carcinoma better predicts survival in patients with stage I/II disease". Surgery. 156 (6): 1378–85, discussion 1385-6. doi:10.1016/j.surg.2014.08.018. PMID 25456914.

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