Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare disease, with a severely adverse prognosis. Clinical reports, even when including a limited number of cases, can contribute to its knowledge. This study aims to characterize patients followed at our department between 1991 and 2013.

Methods: Retrospective analysis of the clinical records of patients with pathological confirmation of ACC. Statistical analysis: SPSS21.

Results: 22 patients (2♂/20♀), 44.5±19.3 years at diagnosis were included. At presentation: abdominal pain (40.8%, n=9), signs of hypercortisolism (31.7%, n=7), asymptomatic (9.1%, n=3). Laboratory: UFC elevation (50%, n=11), testosterone (18.2%, n=4) and S-DHEA (9.1%, n=2). Radiologically: six patients presented invasiveness: kidney (2), diaphragm (2), inferior cava vein (ICV)+kidney (1), ICV+diafragm (1); and eight metastasis: limphatics (4), hepatic (3) and hepatic+lymphatics+pulmonar (1). Twenty were operated: described as complete resection in 90% of the cases; mean tumor weight 740±643 g. Staging (ENSAT): stage II–1 one patients; III–four patients and IV–five patients. Histopathology: incomplete total characterization (median Weiss score ≥3). During follow-up, eight patients presented local recurrence, and 12 new metastasis – lungs (10), liver (5), lymphatics (5) and bone (2). 14 patients were treated with mitotane, in 64.3% (n=9) of the cases after recurrence (initial dose: 2.8±1.8 g/day; maximum dose: 5.6±3.1 g/day and median cumulative dose: 531.5 g, during a median of 9.5 months). The mitotanemia was evaluated in ten patients: 70% (n=7) achieved therapeutic levels. Comparing patients at same stage, those treated with mitotane presented longer survival. One patient had chemotherapy (EDP) associated to mitotane. Median global survival was 11 months (0–257 months), slightly higher in younger adult patients with non-functioning tumors. Seven patients still alive, four of them in remission.

Conclusion: As had been shown in multiple studies of patients with ACC, the best chance of survival may be achieved by an early detection and complete surgical resection of the tumor. Despite the global poor prognosis, we emphasize the long survival of some patients. Although this remains controversial, in this series younger adult age at diagnosis, presence of non-functioning tumor and treatment with mitotane, seems to have allowed some increased survival.