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In the last 12 years, 41 patients with adrenocortical carcinoma were operated on at Cochin Hospital. There were 29 females and 12 males having an average age of 42 +/- 17 years. Seven of them underwent repeat operation for local recurrence after an average interval of 22 months. Eighty per cent of the tumors had secretory activity while 20% did not. Adrenalectomy and lymphadenectomy were performed on all patients. Thirteen (32%) patients underwent extensive resection because of invasive cancer. The same procedure was performed on 7 patients undergoing re-operation. Operative mortality was 4% (2 of 48 patients). Twenty-four patients died of cancer with an average survival time of 22 months (range 2-86 months) and 15 (37%) patients were still alive, 4 of them with metastases, with an average survival time of 51 months. Curative resection for the 31 patients with local (n = 21) or regional disease (n = 10) allowed a 5 year actuarial survival rate of 45%. The 5 year actuarial survival rate for patients undergoing repeat surgery was 33%. Only 1 of the 10 patients with metastases, and who had an impressive response to OP'DDD, was alive 2 years after the operation. The other 9 patients died within 3-4 months after operation. We found that neither patient characteristics or tumor characteristics were of significant prognostic value. There was no overall prolongation of survival in patients receiving OP'DDD. However 4 patients with metastases occurring in the course of their disease experienced an impressive response with OP'DDD and a relatively long survival.