Systemic treatment of advanced-stage adrenal malignancies is most often only palliative. Mitotane alone or in combination with other chemotherapeutic agents such as cisplatin, etoposide, and vincristine are established therapeutic concepts for the treatment of metastatic adrenal cancer. Suramin and gossypol are rarely employed. New therapeutic options are tumor vaccination and treatment with antiangiogenic drugs. Metaiodobenzylguanidine as a radiotherapeutic drug or chemotherapeutic combination therapies that include cyclophosphamide, vincristine, and dacarbazine are applied for systemic treatment of malignant pheochromocytomas.. However, the clinical efficacy of the latter regimen needs further evaluation.