From 1980 to 1998 a series of 265 patients with adrenal tumors underwent surgery, with an adrenocortical carcinoma found in 31 (11.7%). Altogether, 17 (54.8%) patients (group A) had Cushing syndrome (n = 15) or virilization (n = 2), and 14 (45.2%) patients (group B) had nonfunctioning adrenal tumors. Tumor staging was as follows: (groups A/B): stage I, n = 5 (3/2), stage II, n = 14 (9/5), stage III, n = 5 (1/4), stage IV, n = 7 (4/3) patients. There were 12 (38.7%) men and 19 (61.3%) women (median age 51 years, range 25-73 years), and the size of the mass ranged from 3.5 to 20.0 cm (median 8.0 cm), with no differences (p = NS) between groups A and B. Two (6.4%) patients (stage IV) did not undergo surgery and received only palliative drug treatment; 6 (19.4%) were treated with debulking surgery; 15 (48.4%) had unilateral adrenalectomy; and 8 (25.8%) had an extended adrenalectomy. Eighteen (58.0%) patients underwent adjuvant postoperative mitotane treatment, and in 8 (25.8%) patients one or more reoperations for recurrence were required. Nine (29.0%) patients are still alive with a mean follow-up of 34 months; 22 (71.0%) died 2 to 60 months (median 20 months) after surgery. The overall 2- and 5-year survival rates were 62.1% and 10.3%, with no difference (p = NS) between groups A and B. The survival rates at the 1- and 3-year follow-ups were 90.3% and 32.3% (stages I and II) and 71.0% and 6.5% (stages III and IV). In conclusion, adrenocortical carcinoma remains a highly malignant tumor, and stage III-IV patients still have a poor prognosis; but nonfunctioning tumors do not seem to be more aggressive.