Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a very rare neoplasm with an incidence of about one case per million population. It is an agressive tumor (overall 5-year survival 16–38%) and complete resection is the only curative treatment.

Description of methods: We report four cases of ACC. The clinical and biochemical features at diagnosis along with clinical course of the disease are discussed.

Results: Four patients (three female). Mean age at diagnosis 31±14.5 years. Incidentally discovered mass in 50% of cases. CT-scan was the initial imaging test in all cases. Median size 9.23 cm (above 8 cm in all patients). Three patients had functioning tumors (Case 1 hypercortisolism; case 2 hypercortisolism and hyperandrogenism; case 3 hypercortisolism and hyperaldosteronism). Initial Stage was II in three cases and IV in another case. 75% of patients presented local recurrence after surgery. Two of three cases with Stage II at diagnosis developed distant metastases along the clinical course of the disease. 100% of patients with advanced disease had lung metastases. All patients were treated with Mitotane after surgical resection. Three pacients were also treated with chemotherapy (one patient received Etoposide, Doxorubicin and cisplatine; another pacient received Vinorelbine). One patient with bone metastases was treated with palliative radiotherapy. Two patients are still alive (both diagnosed at Stage II). One of them is free of illness alter 41 months. The other patient diagnosed 56 months ago has presented local recurrence and lung and bone metastases. Mean survival in deceased patients was 24±13 months.

Conclusions: ACC are neoplasms with low rate of survival. Stage at diagnosis is related with prognosis. As we can observe in our sample most ACC are functioning. Clinical and radiological features may help in differential diagnosis between benign adenomas and ACC, being size one of the most important predictors of malignancy.