Adrenocortical carcinoma (ACC) is a rare malignant tumor. Early detection is difficult and prognosis is poor. We report on 20 years of ACC surgical experience at our institution.

This study included 32 ACC patients who underwent surgical resection at the Department of Surgery of the Yonsei University Health System in South Korea between January 1990 and February 2012. We reviewed these 32 patients and retrospectively analyzed long-term clinical outcomes and prognosis after radical surgery for ACC.

The median age of the 32 patients at diagnosis was 42.25 years (range 3~81 years). There were 16 (50%) female and 16 (50%) male patients. Mean tumor size was 12.36 cm (range 1.8~20 cm). Twenty-five patients (78.12%) had nonfunctioning tumors while the other seven patients (21.87%) had functioning tumors. Seventeen patients (53.12%) were classified as stage II, two (6.25%) as stage III, and 13 (40.62%) as stage IV. Fourteen patients underwent radical surgical resection, while 14 patients received adjuvant chemotherapy, two received adjuvant radiotherapy, and two received adjuvant chemoradiation. Four patients were lost to follow-up. Among the remaining 28 patients, 15 patients survived. The 5- and 10-year overall survival was 60.6% and 37.8%, respectively (median survival=85±24.3 months). Seventeen patients (53%) experienced disease recurrence. Five- and 10-year recurrence-free survival was 41.5% and 29.7%, respectively (median survival=18±5.5 months).

Early stage at diagnosis and surgical resection were the most important prognostic factors associated with prolonged survival. The role of additional therapy remains controversial and new agents should continually be evaluated for efficacy.