SUN-353 Adrenocortical Carcinoma: A Single Institution Experience in Mexico

Abstract

Background: Adrenocortical carcinoma (ACC) is an exceedingly rare and aggressive cancer, with an incidence of 1-2 cases/ million-year; there are limited ACC data in the Mexican population. Herein we present our experience with ACC at a referral center per a retrospective cohort study. All medical records of consecutive patients with a histological diagnosis of ACC evaluated at our Institution from January, 1st 2000 until August 31st, 2018 were reviewed. Clinical, pathological and laboratory variables were recorded. Results: Sixteen patients with ACC were identified. Median age at diagnosis was 49 years (range, 19-71 years) and 13 were women (81%). Mode of discovery were hormonal excess syndrome (8, 50%) or local symptoms (8, 50%), with none found incidentally. Biochemical proven hormonal excess occurred in 9 (56%), 4 co-secreting corticosteroids and androgens, 2 corticosteroids only, 2 androgens only and 1 aldosterone only. Median tumor size at diagnosis was 12.8 cm (range, 5.9-21 cm) and all had unenhanced CT attenuation >10 Hounsfield units (median HU 33, range 20-39). Initial staging per ENSAT (European Network for the Study of Adrenal Tumors) was: stage II (50%), III (19%) and IV (31%). All patients with localized disease (stage II-III) had a curative intention resection (n=11). For those with advanced disease (stage IV; n=5), cytotoxic chemotherapy was only given to 2 patients. For individuals with initial localized disease, recurrences occurred in 4/11 (36%) and 3 of these were treated with chemotherapy; platinum plus etoposide was the usual regimen. None of the study patients received mitotane, but hormonal blocking therapy was given to 55% (5/9); 4/6 with hypercortisolism. Median follow-up was 13 months (range, 1-139) and at last contact 9 (56%) patients were alive (4 with no evidence of disease). The median overall survival (OS) was 35.5 months (CI 95%, 8.0-62.9). Conclusions: Our data are consistent with other published series; ACC occurs rarely, at ~50 years of age, mostly in women, and commonly as large-sized tumors (~13 cm). Surprisingly none of the cases from our institution were diagnosed incidentally, in contrast to previous studies (~15% incidental). Unfortunately, mitotane was not accessible to our patients given its absence in the Mexican market and patients at our institution are uninsured and cannot gain access to this drug. Nonetheless, the median OS was comparable to the estimated OS of 3-4 years for all ACC patients.