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.