Adrenocortical carcinoma is rare, tends to occur in the first decade as wel
l as the fourth and fifth decades of life, and is slightly more common in w
omen. The tumors are classified as functional or nonfunctional, depending o
n tumor production of corticosteroid, androgen, estrogen, or mineralocortic
oid. Most patients present with large masses and with stage IV disease. Abd
ominal computerized tomography and magnetic resonance imaging are used in t
he evaluation of intraabdominal disease. The most effective treatment for a
drenocortical carcinoma is complete resection. Surgical resection remains t
he only potentially curative treatment for this disease. Early stage and cu
rative resection are the two clinical factors that are of prognostic signif
icance for long-term survival. Mitotane is the chemotherapeutic agent most
often used to treat adrenocortical carcinoma. Its efficacy in prolonging su
rvival is limited but may be: enhanced by monitoring of serum levels and th
eir 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.