[Adrenocortical carcinomas: therapeutic advances in 2011]

Ann Endocrinol (Paris). 2011 Oct;72 Suppl 1:S8-S14. doi: 10.1016/S0003-4266(11)70004-X.
[Article in French]


Adrenocortical cancer (ACC) is a rare aggressive malignancy with a poor prognosis (5-year survival: 45 %). Their management requires multidisciplinary expertise. Complete resection by an expert surgeon is the only curative treatment. Very few adjuvant treatments are available and their efficacy is not fully proved. Adjuvant mitotane therapy increases the disease-free survival in the majority of patients after surgery but further studies are needed to determine patients in whom this treatment is the more beneficial. Blood concentrations of mitotane between 14 and 20mg/l are necessary to have a full efficiency but this therapeutic window may cause side effects difficult to control. When aggressive parameters are present, radiotherapy is proposed. In case of residual or unresectable disease, combination of chemotherapy and mitotane is conventionally proposed. FIRM-ACT study establishes that EDP (etoposide, doxorubicine et cisplatine) is the most effective chemotherapy for progressive ACC. The first results of treatment with tyrosine kinase inhibitors, in patients with progressive disease despite one or two lines of chemotherapy are disappointing but this may be partly explained by the interaction with mitotane which reduces the plasma concentrations of sunitinib in particular. Clinical trials are underway to assess the effectiveness of other targeted therapies, including treatments acting on the IGF-1 receptor.

Publication types

  • English Abstract

MeSH terms

  • Adrenal Cortex Neoplasms / drug therapy*
  • Adrenal Cortex Neoplasms / radiotherapy
  • Adrenal Cortex Neoplasms / surgery
  • Adrenocortical Carcinoma / drug therapy*
  • Adrenocortical Carcinoma / radiotherapy
  • Adrenocortical Carcinoma / surgery
  • Antineoplastic Agents / adverse effects
  • Antineoplastic Agents / therapeutic use*
  • Antineoplastic Combined Chemotherapy Protocols
  • Cisplatin
  • Clinical Trials as Topic
  • Combined Modality Therapy
  • Disease-Free Survival
  • Epirubicin
  • Humans
  • Mitotane / adverse effects
  • Mitotane / blood
  • Mitotane / therapeutic use*
  • Protein Kinase Inhibitors / therapeutic use
  • Taxoids
  • Treatment Outcome


  • Antineoplastic Agents
  • Protein Kinase Inhibitors
  • Taxoids
  • Epirubicin
  • Mitotane
  • Cisplatin

Supplementary concepts

  • EDP protocol