Adrenocortical carcinoma: retrospective study of 14 patients experienced at a single institution over 34 years.

Objective: To review clinical outcome of patients with adrenocortical carcinoma experienced at a single institute over 34 years.

Methods: The study included 14 patients who were diagnosed as having the disease and were treated at the Department of Urology, Sapporo Medical University Hospital between 1973 and 2006. Their clinical features and outcomes were reviewed.

Results: Of the 14 patients, there were nine men and five women. The median follow-up period was 13.0 months (range, 1-213). Two patients were classified as having stage II disease, seven as stage III and five as stage IV. The disease was completely removed in eight patients and incompletely in three. Two other patients received exploratory laparotomy only. The remaining one patient had no indication for surgery. The median survival periods were 2 months in patients with stage IV and 108 months in those with stages II and III (P = 0.136). Mitotane treatment in the adjuvant setting did not clearly affect the clinical courses of patients without metastasis. However, the treatment was effective for metastasis that was repeatedly developed as late recurrence in one patient. Three patients with metastasis at diagnosis received combination chemotherapy with etoposide, doxorubicin and cisplatin (EDP) with or without mitotane treatment, to which lung metastasis completely responded in one patient.

Conclusions: Adrenocortical carcinoma is a rare disease but frequently recurs. The best chance of survival may be achieved by early detection and complete surgical removal. There may be patients who possibly benefit from mitotane treatment with or without EDP, although this remains to be conclusively determined.