Emerging drugs for adrenocortical carcinoma

Expert Opin Emerg Drugs. 2008 Sep;13(3):497-509. doi: 10.1517/14728214.13.3.497.


Background: Adrenocortical carcinoma (ACC) is an extremely rare aggressive disease. Few data are available on the efficacy of systemic antineoplastic treatments (mitotane and cytotoxic therapy) in the treatment of advanced disease.

Objective/methods: this paper will review the existing treatment strategies and new perspectives in the management of ACC patients.

Results/conclusion: An ongoing randomized international trial aims to define the best combination chemotherapy plus mitotane regimen. Based on the results of a case control study, mitotane is being explored as adjuvant therapy. Genetic and biological studies have identified molecular targets for specific targeted drugs such as IGF receptor inhibitors and antiangiogenetic drugs. Phase II trials are exploring the activity of these drugs either alone or in combination with chemotherapy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenal Cortex Neoplasms / drug therapy*
  • Adrenocortical Carcinoma / drug therapy*
  • Antineoplastic Agents, Hormonal / administration & dosage
  • Antineoplastic Agents, Hormonal / therapeutic use*
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Chemotherapy, Adjuvant
  • Humans
  • Mitotane / administration & dosage
  • Mitotane / therapeutic use*


  • Antineoplastic Agents, Hormonal
  • Mitotane