Endocrine Abstracts

Background: Adrenocortical carcinoma (ACC) is a rare and aggressive neoplasm with a 5-year survival rate <30%. Survival depends mainly on the stage at diagnosis, being surgery the only curative treatment.

Objective: To report the clinical features, management and outcomes of a series of ACC in two Spanish tertiary hospitals.

Material and methods: All patients with pathological diagnosis of ACC between 2000 and 2017 were included in this study. We herein report on data from clinical features, hormonal functionality, imaging, European Network for the Study of Adrenal Tumors (ENSAT) staging at diagnosis and response to treatment.

Results: We evaluated 28 patients (15 women, 53.6%) with a median age of 54.8 years [36–80]. Median tumor size was 12.1 cm [4–26]. Ten patients (35.7%) had hormonal overproduction: androgens in 10 patients, cortisol in 8 and mineralocorticoid in one. At diagnosis there were 16 patients (57.1%) in stage IV, 8 (28.6%) in stage III, 8 (28.6%) in stage III and 4 (14.3%) in stage II. Four patients presented vena cava thrombosis. Surgery was performed in 26 patients, 20 of them with a complete resection. Twenty-four patients received mitotane (9 patients with concomitant chemotherapy, mainly cisplatin/etoposide, and one with concomitant chemoradiotherapy). Thirteen patients (54.2%) in stage IV, 7 (29.2%) in stage III and 4 (16.6%) in stage II. Thirteen out of 24 that received mitotane had disease progression and 9 died (8 in stage IV and one in III). Median daily dosage of mitotane was 2 g/day [1–10], with median treatment duration of 16 months [0–116]. Eleven patients (45.8%) suffered some adverse effect due to treatment and in 2 cases mitotane was withdrawn. Plasma mitotane levels were measured in 16 patients (66,7%). Appropriate therapeutic levels of mitotane were achieved in 57.9% of the measurments in 11 patients. Median follow-up of the patients was 27 months [0–316]. Eleven patients died: 9 in stage IV and 2 in III (6 of them with hypercortisolism). Two patients were lost for follow-up. In the group of the 15 surviving patients, 5 were in stage II at the diagnosis (one developed metastasis), 6 in III and 4 in IV.

Conclusions: One third of our ACC series were hyperfunctional, most of them multisecretory. Hypercortisolism could be a bad prognostic factor as well as the stage at the diagnosis. Mitotane can be a useful tool, but is mandatory to monitor plasma levels in order to get an effective response.