Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy, with an incidence of approximately 0.72 per million cases per year leading to 0.2% of all cancer deaths in the United States. Metastatic ACC has a dismal prognosis with an overall survival of less than 1 year. We present a case of a 37-year-old man with metastatic ACC with unusual good prognosis and review the therapeutic options in the literature.
Keywords: Adrenocortical carcinoma; Chemotherapy; Mitotane.