ADRENAL-CORTICAL CARCINOMA - EPIDEMIOLOGY AND TREATMENT WITH MITOTANEAND A REVIEW OF THE LITERATURE

Background. Adrenal cortical carcinoma is rare; the authors have treat ed only eight patients with the disease at Good Samaritan Regional Med ical Center since 1974. No exhaustive collection of cases of this canc er has been done since 1952. Methods. The authors retrospectively revi ewed the medical records of their eight patients with adrenal cortical carcinoma. They also searched the English literature from 1952 to 199 2 for reports of patients with the disease. They treated each report a s a series if two or more previously unreported patients were reported . They paid special attention to patients for whom stage of disease wa s noted at diagnosis, treatment with mitotane (o,p'-DDD) was used, and the outcome was reported. Results. Five were male and three were fema le patients. Five had nonfunctional tumors. None were pediatric. The a uthors found 1891 cases in the English literature. Adrenal cortical ca rcinomas are more common in women (58.6%) than in men (41.4%). The age distribution of tumors is bimodal, with peaks in the first and fifth decades. Tumors in children are more commonly functional (83.5% in fem ale patients, 85.6% in male patients), although nonfunctional tumors a re more common in older patients (84.7%). Most (68%) of these tumors a re diagnosed late in disease when surgery is no longer curative. Only 35% of patients treated with mitotane had a clinical response. Conclus ions. Adrenal cortical carcinomas are diagnosed most often in children because of functionality and older men because of mass effect. Most t umors are discovered too late for curative resection. Treatment of met astatic disease with mitotane has limited success.