Seventy-seven patients with histologically proven adrenal cortical carcinoma seen at the University of Texas M. D. Anderson Hospital (1950-1981) were studied. Thirty-nine were women (mean age at diagnosis, 36.6 years), and 38 were men (mean age at diagnosis, 48.3 years). On presentation, 41 of 74 had abdominal symptoms (55.4%) and 25 of 74 had an abdominal mass (33.8%). Twenty-six patients (33.8%) were found to have clinically functional disease (18 women, 8 men). At diagnosis, 26 (33.8%) had clinical or radiologic signs of distant metastases. Preoperative radiologic studies yielded an abnormal intravenous pyelogram in 42 of 51 (82%), an abnormal abdominal ultrasound in eight of nine (88.9%), abnormal computerized tomography in 10 of 10 (loo%), and abnormal arteriogram in 18 of 19 (94.7%). Surgery for localized or regional disease was associated with a disease-free interval of at least 2 years in 16 of 34 patients (47%). The use of op'DDD, abdominal radiotherapy, and systemic chemotherapy produced demonstrable effects in nine of 47 (19.1%), two of nine (22.2%), and three of 26 (1 IS%), respectively. Distant metastases occurred in 60 patients, commonly in lung, liver, peritoneal and pleural surfaces, lymph nodes, and bone. Analysis of survival data showed a 5-year survival of approximately 30%. The authors concluded that early diagnosis and radical surgery offer the only prospects of long-term survival and the possibility of cure.