Adrenocortical carcinoma is rare, tends to occur in the first decade as well as the fourth and fifth decades of life, and is slightly more common in women. The tumors are classified as functional or nonfunctional, depending on tumor production of corticosteroid, androgen, estrogen, or mineralocorticoid. Most patients present with large masses and with stage IV disease. Abdominal computerized tomography and magnetic resonance imaging are used in the evaluation of intra-abdominal disease. The most effective treatment for adrenocortical carcinoma is complete resection. Surgical resection remains the only potentially curative treatment for this disease. Early stage and curative resection are the two clinical factors that are of prognostic significance for long-term survival. Mitotane is the chemotherapeutic agent most often used to treat adrenocortical carcinoma. Its efficacy in prolonging survival is limited but may be enhanced by monitoring of serum levels and their maintenance at elevated values. Even for patients who undergo complete resection, recurrent and metastatic disease are extremely common. The only effective treatment for recurrent disease is reoperation.
