Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature.

BACKGROUND: Adrenal cortical carcinoma is rare; the authors have treated only eight patients with the disease at Good Samaritan Regional Medical Center since 1974. No exhaustive collection of cases of this cancer has been done since 1952.
METHODS: The authors retrospectively reviewed the medical records of their eight patients with adrenal cortical carcinoma. They also searched the English literature from 1952 to 1992 for reports of patients with the disease. They treated each report as a series if two or more previously unreported patients were reported. They paid special attention to patients for whom stage of disease was noted at diagnosis, treatment with mitotane (o,p'-DDD) was used, and the outcome was reported.
RESULTS: Five were male and three were female patients. Five had nonfunctional tumors. None were pediatric. The authors found 1891 cases in the English literature. Adrenal cortical carcinomas 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 female patients, 85.6% in male patients), although nonfunctional tumors are more common in older patients (84.7%). Most (68%) of these tumors are diagnosed late in disease when surgery is no longer curative. Only 35% of patients treated with mitotane had a clinical response.
CONCLUSIONS: Adrenal cortical carcinomas are diagnosed most often in children because of functionality and older men because of mass effect. Most tumors are discovered too late for curative resection. Treatment of metastatic disease with mitotane has limited success.