Adrenocortical carcinoma is a rare and aggressive malignancy with an incidence of about 1 to 2 cases per 1 million people per year and a median survival of 17 months.1 Surgical resection is the treatment most likely to prolong survival, but many patients have a relapse or present with or develop disseminated metastases for which systemic therapy is required. Mitotane (1-[o-chlorophenyl]-1-[p-chlorophenyl]-2,2-dichloroethane or o,p’-DDD)—a compound that inhibits enzymes involved in adrenocortical steroid synthesis but has low cytotoxic activity against adrenocortical carcinoma—and the chemotherapy drug cisplatin alone, or in drug combinations, offer the greatest efficacy for the treatment of advanced disease.