Adrenocortical carcinoma – our experience with 11 cases

Abstract.

Background and aims: Adrenocortical carcinoma (ACC) is a rare tumour with an incidence of approximately 0.5–2 cases per million per year. Diagnosis is mostly delayed and prognosis is poor. We report our experiences with 11 patients operated on within the last 10 years. Patients/Methods: The data of the patients with ACC were reviewed and presenting symptoms, diagnostic procedures, treatment and results of follow-up were evaluated. Results: The group of patients consisted of eight women and three men with a mean age of 40.2 (15–57) years. Median follow-up was 16 (1–71) months. Six patients presented with Cushing's syndrome, two presented with virilism and hirsutism caused by androgen-producing tumours. Three patients had hormonally inactive tumours. At the time of diagnosis, five tumours were classified as stage II, two as stage III and four as stage IV. Four patients had tumours with intravascular extension, prompting recurrence in two cases. Eight adrenalectomies, one resection of local recurrence, one adrenalectomy with splenectomy and one adrenalectomy and resection of a liver metastasis were performed. Five patients received additional chemotherapy. Five of the 11 patients are still alive (three stage II, one stage III and one stage IV at the time of diagnosis), three of whom have no evidence of disease (14, 48 and 71 months after surgery). The other six patients died after a median postoperative period of 10 (1–21) months. Conclusions: Venography should be performed prior to surgery to detect or exclude thrombotic tumour masses in the suprarenal vein, renal vein or inferior vena cava. Radical surgery is the only curative approach and is recommended for all patients with resectable tumours, including those patients with recurrent disease. There is no consensus concerning adjuvant therapy. The value of multidisciplinary strategies needs to be assessed in multicentre trials.