Adrenocortical carcinoma: what you at least should know

Adrenocortical carcinoma (ACC) is rare, with an incidence of 0.5-2 per million per year.ACC usually occurs in the fifth or sixth decade 1 with a slight female predominance (sex ratio 1.5 : 1).Most cases are sporadic, but ACC has also been associated with some hereditary syndromes including multiple endocrine neoplasia type 1, Li-Fraumeni syndrome, Lynch syndrome, familial adenomatous polyposis, and Beckwith-Wiedemann syndrome.The prognosis of ACC is heterogeneous, with median overall survival of 3 years.Surgery remains the only hope for cure 2 .Diagnosis may be challenging especially when faced with an adrenal incidentaloma.Its management requires a multidisciplinary approach that includes at least surgeons, endocrinologists, pathologists, nuclear medicine physicians, and radiologists.Here, the authors present what surgeons should at least know to provide the highest standard of care to patients with ACC, as summarized in Fig. 1.