Adrenocortical carcinoma: a practical guide for clinicians

Adrenocortical carcinoma is a rare endocrine malignancy. The management of patients with adrenocortical carcinoma is challenging for several reasons, including its heterogeneous but frequently aggressive biological behaviour; tumour-related hormonal excess (eg, Cushing's syndrome or virilisation); the overall paucity of evidence regarding diagnostic investigation and treatment; the approval of only one drug (mitotane); and the scarcity of centres with sufficient experience. In this Review, we present 25 questions on the most important aspects of the clinical management of adult patients with adrenocortical carcinoma that we have frequently asked ourselves over the past 25 years. We offer our personal answers and perspectives, drawing upon published evidence as well as more than 60 years of collective clinical experience and insights from our management of more than 1700 patients across two centres in Germany and Italy.