Adrenocortical carcinoma: The management of metastatic disease

• The management of metastatic ACC is challenging and effective systemic treatments are lacking.
• The molecular characterization of ACC may help to understand the pathogenesis and to define novel therapeutics.
• In this manuscript we reviewed the advances in the therapy of advanced ACC.

Adrenocortical cancer is a rare malignancy. While surgery is the cornerstone of the management of localized disease, metastatic disease is hard to treat. Cytotoxic chemotherapy and mitotane have been utilized with a variable degree of benefit and few long-term responses. A growing understanding of the molecular pathogenesis of this malignancy as well as multidisciplinary and multi-institutional collaborative efforts will result in better defined targets and subsequently, effective novel therapies.