Systemic Therapy in Locally Advanced or Metastatic Adrenal Cancers: A Critical Appraisal and Clinical Trial Update.

Mitotane and chemotherapy with etoposide, doxorubicin, and cisplatin plus mitotane (EDP-M) are the only therapies with demonstrated efficacy in advanced adrenocortical carcinoma. Prognostic and predictive factors are needed to identify patients who could obtain the best benefit from these treatments. Despite the strong rationale for their use, clinical trials on molecular targeted therapies have failed to demonstrate that these drugs are efficacious in the management of this extremely rare disease.