Management of adrenocortical carcinoma

J Natl Compr Canc Netw. 2009 Jul;7(7):752-8; quiz 759. doi: 10.6004/jnccn.2009.0051.


Adrenocortical carcinomas (ACCs) are rare tumors that arise from the cortex of the adrenal gland with an incidence 1 to 2 per million. The rarity of this tumor translates into a paucity of experience in managing patients in most medical centers. Because clinical series are small and prospective evaluation of treatment strategies is limited, the current state of knowledge is strongly influenced by expert consensus opinion from a few medical centers specializing in ACCs. This article describes the basic diagnostic and prognostic issues in adrenal cancer management, and presents detailed rationales for therapeutic management.

MeSH terms

  • Adrenal Cortex Neoplasms / diagnosis
  • Adrenal Cortex Neoplasms / epidemiology
  • Adrenal Cortex Neoplasms / therapy*
  • Adrenocortical Carcinoma / diagnosis
  • Adrenocortical Carcinoma / epidemiology
  • Adrenocortical Carcinoma / therapy*
  • Antineoplastic Agents, Hormonal / therapeutic use
  • Catecholamines / analysis
  • Chemotherapy, Adjuvant
  • Cushing Syndrome / drug therapy
  • Cushing Syndrome / etiology
  • Diagnostic Imaging
  • Humans
  • Hydrocortisone / analysis
  • Metanephrine / analysis
  • Mitotane / therapeutic use
  • Neoplasm Metastasis
  • Neoplasm Staging
  • Prognosis


  • Antineoplastic Agents, Hormonal
  • Catecholamines
  • Metanephrine
  • Mitotane
  • Hydrocortisone