Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a very rare entity with a variable clinical presentation.

Objective: To review the casuistry of ACC in our center in the last 15 years and to assess clinical characteristics and evolution in our patients.

Material and method: We searched cases of ACC from 2004 to December 2018 registered in the Pathology Department.

Results: Fifteen cases were found, 9 women and 6 men. The mean age at diagnosis was 49.6 ± 12.7 years (range 27–82 years). The initial symptom was local discomfort (5), clinical hypercortisolism (4) constitutional syndrome (2), fortuitous (3), and unknown (3). Regarding location 8 were located on the right and 6 on the left gland. The size of the lesion at diagnosis was 11.15 ± 4.1 cm (range: 5.4–21 cm). Cortisol production was found in 7 cases. At the time of diagnosis, 4 had metastases (liver and/or lung). Only one case could not be intervened due to disseminated disease. After surgery, 5 developed local recurrence and 3 distant metastases. For 2 patients we do not have data regarding post-surgical evolution. Regarding need for post-surgical treatment: 4 patients were not treated for low aggressiveness, 1 for disseminated disease at diagnosis and 1 for post-operative death. 1 patient received adjuvant treatment with Mitotane and 5 Mitotane+etoposide, doxorubicin, and cisplatin (EDP). Only 3 patients have cure criteria today after more than 10 years of diagnosis, 7 patients with a mean survival of 9.3 months (range 3–12) have died, 1 patient has lost follow-up and 4 follow-up reviews of which 2 of them have metastatic disease (at 4 and 20 months post-surgery) and the other 2 are free of disease at this time (at 7 and 38 months post-surgery).

Conclusions: ACC is a very infrequent and aggressive entity in which is necessary to maintain an important degree of alertness and, above all, multidisciplinarity.