Endocrine Abstracts

Background: Adrenal cortical carcinoma (ACC) is a rare malignancy known to be highly aggressive, with few specific treatment options. The objective was to evaluate clinicopathological features and outcome in patients treated at Helsinki University Hospital

Methods and patients: We included all patients diagnosed with adrenocortical carcinoma (Weiss score ≥4) at Helsinki University Hospital during the years 2002 through 2017. Patients were identified from our electronic patient records using ICD-10 codes C74.0 and 74.9 and from the Pathology register. Data on presentation, surgical and medical treatments were retrieved from patient records. Radiologic and histopathological characteristics were re-evaluated.

Results: Forty-two patients were diagnosed and treated for ACC, five of which were children. Of the adult patients, 23 (62%) were women, mean age at diagnosis was 56 years (18–84). Median follow-up was 41 (1–199) months. Twenty-seven (75%) of the tumours were found incidentally. Abdominal pain was the most common symptom, occurring in 11/36 (31%) of the patients, 6/36 (17%) presented with hirsutism, 5/36 (14%) had bruises, and 2/23 (9%) menstrual disturbances. Fifty-four percent (19/35) had biochemically verified hypercortisolism, 19% (5/27) hyperaldosteronism and hyperandrogenism was confirmed in all the 11 females (48%) that were investigated for hyperandrogenism. According to ENSAT staging, 6/36 (17%) had stage I, 13/36 (37%) stage II, 8/36 (22%) stage III and 9/36 (25%) stage IV disease. Mean tumour size was 95 mm (20–196 mm) and, in 67% (24/36) the origin was the left adrenal. Mean Hounsfield units were 33 HU (21–45), mean Ki67 17% (1–40%), mean Weiss score 7.1 (4–9) and Helsinki score 24 (4–48). Thirty-three (89%) patients underwent primary surgery, metastases were resected in 4. Thirty (81%) received adjuvant mitotane therapy, therapeutic concentrations were reached in 18 (60%) in a mean of 334 days (78–1055), with a mean cumulative dose of 1030 g (168.5–1847.5). Treatment was discontinued in 9 (30%) patients because of adverse effects. Further oncological treatments were given to 35% (13/37). The 5-year survival rate was 67%.

Conclusion: To date, ACC often presents as an incidental finding. In contrast to benign incidentalomas, ACC is always characterized by a high HU (> 20) on non-contrast CT. Of the patients, 31% presented with abdominal pain and 54% had biochemically verified hypercortisolism. In this cohort, 83% had at least stage II disease and the 5-yr survival rate of 67% was better than in most previous reports.