An eleven-year experience with adrenocortical carcinoma.

BACKGROUND: Key issues in the treatment of adrenocortical carcinoma are the value of adjuvant therapy, the value of reoperation, and the search for effective chemotherapeutic agents. The present series was reviewed to evaluate these issues.
METHODS: We present a retrospective series of 73 patients with adrenocortical carcinoma treated at a single institution.
RESULTS: Twenty patients had carcinomas that were unresectable, and 53 patients underwent complete resections. Ten patients received adjuvant therapy (mitotane, seven patients; radiation, three patients). Forty-five (85%) patients had recurrence, including all who received adjuvant therapy. Mean disease-free intervals for those who did and did not receive adjuvant therapy were equivalent at 2.4 years. Nineteen patients with recurrent disease received chemotherapy, and 26 patients underwent 51 reoperations to resect recurrent and metastatic disease. The overall 5-year survival rate, which was 35%, was 47% for patients with complete resection. Stage and resectability were prognostic factors. Mean survival time for patients with recurrent disease treated medically was 19 months compared with 56 months for patients who underwent reoperation. Mitotane had a 24% partial response rate. Other chemotherapeutic agents were ineffective.
CONCLUSIONS: We conclude that an aggressive surgical approach to recurrent and metastatic disease should be adopted and that patients should be resected free of disease whenever possible. Currently no effective chemotherapy exists, and the value of adjuvant therapy remains unproved.