Introduction: Adrenocortical carcinomas are rare neoplasms with an incidence of 0.7 to 1.5 per million/year. Diagnosis often requires a multidisciplinary approach. When the disease is confined to the adrenal gland, absolute criteria defining malignancy, do not exist, however a score of Weiss> or =a 3 is considered diagnosis. The prognosis is usually reserved and conventional chemotherapy is not curative.
Material and Methods: Retrospective analysis of the clinical and demographic characteristics, review of the anatomopathological and therapeutic criteria of patients with adrenocortical carcinoma treated at our Institution between 1999 and 2015.
Results: Twenty-two patients with a mean age of 56 years were evaluated, 50% of cases were male. The majority of patients (63.6%) were symptomatic and pain was the most frequent symptom (57.1%). All patients underwent surgical treatment. Multiorgan resection was performed in 5 cases (22.6%). The surgery was R0 in 72.7%. After histological examination, 72.7% presented Weiss score> 2. Adjuvant therapy was performed in 5 patients (22.6%). Considering the revision of the Weiss score, TNM staging and margins status, 50% of the patients would had indication for adjuvant treatments. In this series, the relapse rate (local or distance) was 38.1% (8 cases) and the overall survival at 5 years was 62%; which compares favorably with that described in the literature.
Conclusions: The rarity of this pathology makes diagnosis and therapeutic orientation difficult. A rigorous and standardized histological evaluation is imperative to ensure the most appropriate adjuvant treatment. The referral of this patients to centers of expertise is essential, with the hope that new target therapies will lead to better results.
20 - 23 May 2017
European Society of Endocrinology