Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare and aggressive tumor that accounts for 0.2% of cancer-related deaths. Case series and prospective studies are very limited due to its low prevalence. Current knowledge is based primarily on the opinions of experts in specialized units. The aim of this study is to perform a descriptive analysis of the management and prognosis of patients with ACC in HCSC in the last 20 years.

Material and methods: Eighteen cases of ACC between 1997 and 2017 in HCSC were retrospectively reviewed. All data were analyzed including demographic, epidemiological and clinical information using SPSS 15.0. The study obtained the approval of the local Ethics Committee.

Results: 11/18 (61%) were male and the mean age was 54.8 (S.D. 15.3). Diagnosis was incidental in 5/18 (27.8%). The most common presentation (6/18, 34.6%) was constitutional symptoms. 39% of the tumors (7/18) were functioning; 2 patients presented Cushing’s syndrome, 2 hyperandrogenism, 2 presented with both and 1 with hyperaldosteronism. In 2/18 (11%) serum hormone levels were not performed. 4/18 (22%) were never evaluated by an endocrinologist. All patients had CT performed, 5/18 (28%) had MRI, 4/18 (22%) had PET and MIBG-scintigraphy, and 7/18 (39%) had FNA. The mean size was 118.4 (S.D. 69.9) mm. 13/18 (72%) were located on the left side. Upon diagnosis, 9/18 (50%) and 5/7 (71.4%) of the functioning were in stage IV. 16/18 (89%) had surgery (open in 14/18, 87%). 12/18 (67%) received mitotane (between 1 and 57 months), whose plasma levels were monitored only in half of them. It was discontinued in 3/12 (25%) due to adverse effects. 7/18 (39%) received different lines of chemotherapy, 7/18 (39%) targeted therapies, 3/18 (16.6%) radiotherapy and 1/18 (5.5%) chemoembolization. 5/18 (28%) received only palliative care. 14/18 (78%) are deceased. Median survival time was 41 months (min 2 – max 92) with no significant differences according to stage (except stage I), functionality or use of mitotane therapy.

Conclusions: In our centre, ACC was an uncommon tumor with poor prognosis regardless of sex, stage (except in stage I), treatment applied or tumor functionality. It was usually diagnosed in middle-aged patients and in advanced stages. The most common treatments used were surgery and mitotane. We also noticed that there is a considerable lack of standardization in the treatment strategy of this tumor for advanced cases in our centre. Patients with ACC should be treated in highly specialized units by a multidisciplinary team.