The Cleveland Clinic experience with adrenal cortical carcinoma.

Between 1936 and 1987, 82 patients with adrenal cortical carcinoma were seen at our clinic. Of these patients 49 (72 per cent) have been seen during the last 25 years. A total of 40 patients (48.8 per cent) presented with a hormonally functional tumor and 42 (51.2 per cent) had a nonfunctional tumor. Forty patients (48.8 per cent) presented with localized disease, 12 (14.6 per cent) with regional disease and 30 (36.6 per cent) with distant metastases. Complete surgical removal of all gross tumor was achieved in 49 patients. Over-all 3 and 5-year patient survival rates in this series were 37.5 and 25.1 per cent, respectively. Survival was significantly improved (43.9 per cent at 5 years, p equals 0.0001) in patients with localized disease that was completely removed surgically; postoperative adjuvant therapy with op'-DDD was of no benefit in these patients. Survival in patients with metastatic disease was poor and was not improved by treatment with op'-DDD, cytotoxic chemotherapy or radiation therapy.