Adrenocortical carcinoma (ACC) is an aggressive cancer characterized by poor survival. Apart from radical surgery, there is a limited range of therapeutic options and mitotane remains the cornerstone of medical treatment of ACC in either adjuvant or palliative settings. The aim of adjuvant mitotane therapy is to reduce the risk of ACC recurrence following surgical removal of the tumor. Use of mitotane in an adjuvant setting is off-label, but the recent guidelines endorsed by the European Society of Endocrinology (ESE) and the European Network for the Study of Adrenal Tumors (ENSAT) recommend it in ACC patients at high risk of recurrence. The palliative use of mitotane for treatment of advanced ACC aims at controlling tumor progression and, when present, hormone secretion. In this clinical setting, mitotane is used in association with chemotherapy to treat the more aggressive forms, while mitotane monotherapy is reserved for less progressive ACC. Many years after its introduction in clinical practice, there are still uncertainties surrounding the use of this old drug and the derived benefits. Moreover, physicians who use mitotane should recognize and manage the systemic effects of the drug that need a complex supporting therapy.

肾上腺皮质癌（ACC）是一种以生存期较差为特征的侵袭性癌症。除根治性手术外，治疗选择范围有限，在辅助或姑息治疗中，米线烷仍是ACC药物治疗的基石。辅助线粒体治疗的目的是降低手术切除肿瘤后ACC复发的风险。在佐剂环境中使用米诺坦是不可行的，但是欧洲内分泌学会（ESE）和欧洲肾上腺肿瘤研究网络（ENSAT）认可的最新指南建议在高复发风险的ACC患者中使用它。mitotane用于晚期ACC的姑息治疗旨在控制肿瘤的进展以及激素分泌（如果存在）。在这种临床情况下，mitotane与化学疗法结合使用可治疗更具侵略性的形式，而mitotane单一疗法则保留用于进行性较差的ACC。在将其引入临床实践多年之后，围绕这种旧药物的使用及其产生的益处仍然存在不确定性。此外，使用米诺坦的医生应认识并管理需要复杂支持疗法的药物的全身作用。