Adrenocortical carcinoma is a rare malignant tumor of poor prognosis, frequently requiring additional treatments after initial surgery. Due to its adrenolytic action, mitotane has become the first-line medical treatment in patients with aggressive adrenocortical carcinoma. Over the last 2 years, apart from the classical chemotherapy based on etoposide and platinum salts, several studies reported the use of drugs such as temozolomide, tyrosine kinase inhibitors or immunotherapy, with more or less convincing results. The aim of this review is to give further insights in the use of these drugs, and to describe potential therapeutic perspectives based on recent pangenomic studies, for the future management of these still difficult to treat tumors.

肾上腺皮质癌是一种罕见的恶性肿瘤，预后不良，初次手术后经常需要额外的治疗。由于其肾上腺素溶解作用，米托坦已成为侵袭性肾上腺皮质癌患者的一线药物。近 两年，除了依托泊苷和铂盐的经典化疗外，多项研究报道了使用替莫唑胺、酪氨酸激酶抑制剂或免疫疗法等药物，或多或少的令人信服的结果。本综述的目的是进一步深入了解这些药物的使用，并根据最近的泛基因组研究描述潜在的治疗前景，以便未来管理这些仍然难以治疗的肿瘤。