BACKGROUND

Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis. The clinical experience acquired, even from a small number of cases, has improved understanding of this condition. The purpose of this study is to characterize patients with ACC followed up at a Portuguese reference center over the past 22 years.

METHODS

Retrospective analysis of clinical records of patients with histopathological diagnosis of ACC followed up from 1992 to 2014.

RESULTS

The study sample consisted of 22 patients, 20 of them female. Eleven patients were in stage II, four in stage III, and five in stage IV; 13 patients had functioning lesions. Adrenalectomy was performed in 20 patients, with complete tumor resection in 90% of the cases. During follow-up, eight patients experienced recurrence of local disease, and 12 distant metastases. Fourteen patients received mitotane, 35.7% (n=5) as adjuvant therapy and 64.3% (n=9) after recurrence; therapeutic plasma mitotane levels were achieved in 70% of patients. Stage III patients who received adjuvant therapy had longer survival time (13.5 vs. 2.5 months). Two patients were given chemotherapy associated to mitotane. Median survival was 11 months (0-257 months); it was slightly longer in younger patients or patients with non-functioning tumors. Six patients are still alive, four of them with no evidence of disease.

CONCLUSION

Despite the overall poor prognosis, some patients with ACC may have a long survival time. Although complete tumor removal remains the only potentially curative treatment, diagnosis at a younger age, presence of non-functioning tumors, and mitotane treatment also seemed to be associated to longer survival in our patients.