Adrenocortical carcinoma is a rare cancer with a dismal prognosis, which affects 1–2 people per million annually, worldwide.1 Because of the small number of patients within single centres and the scarcity of preclinical models until recently,2 advancements in treatment options have been limited. Mitotane, a cell-specific adrenolytic, has been the mainstay of treatment, together with etoposide, doxorubicin, and cisplatin chemotherapy.3 Early studies with tyrosine kinase inhibitors (TKIs) have not shown a strong signal of response in patients with adrenocortical carcinoma as monotherapy.