SPECIAL COMMENTARIESTreatment of adrenocortical carcinoma: oncological and endocrine outcomesBrönimann, Stephan; Garstka, Nathalie; Remzi, Mesut Author Information Department of Urology, Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria Correspondence to Mesut Remzi, MD, Department of Urology, Vienna General Hospital, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria. Tel: +43 1 40400 26150; fax: +43 1 40400 32230; e-mail: [email protected] Current Opinion in Urology 33(1):p 50-58, January 2023. | DOI: 10.1097/MOU.0000000000001045 Buy Metrics Abstract Purpose of review Adrenocortical carcinoma (ACC) is a rare, aggressive disease with a paucity of data and great variability between published studies regarding its treatment. This review provides information on current clinical management and oncological and endocrine outcomes. Recent findings Complete surgical resection is the only potentially curative treatment for adrenocortical carcinoma (ACC). Adjuvant mitotane treatment is recommended in patients with favourable/intermediate prognosis. As part of the endocrine follow-up, steroid hormones and thyroid hormones may be decreased or increased and may need to be substituted or suppressed. Recurrences are common. If the disease-free interval is more than 12 months, surgery is a treatment if complete resection is feasible. In advanced/metastatic ACC patients, the prognosis is poor. Mitotane monotherapy is only appropriate for patients with low tumour burden and indolent disease. Patients with unfavourable prognosis should be treated with aggressive cytotoxic therapy. Patients requiring third-line treatment should be considered for clinical trials. Immunotherapy and targeted therapy are currently being investigated, but have so far yielded only unsatisfactory results. Summary There is scarce evidence for the treatment of ACC, which often complicates clinical decision-making. Patients who progress on EDP-M should be treated in clinical trials. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.