Clinical and molecular prognostic factors in adrenocortical carcinoma

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare cancer, with an incidence less than 0.7-1.5 per 1 million people per year, with a poor prognosis. The overall survival (OS) depends on the ENSAT stage: in particular in metastatic ACC the OS varies from 10 to 20 months, with a 5-year survival around 10%. ACC has a different behavior, probably due to a different biology. For this reason, a careful prognostic classification is mandatory, in order to stratify the patients and propose a specific management. EVIDENCE ACQUISITION: Prognostic factors can be divides in three groups: clinical factors (tumor stage, age, hormone-related symptoms), pathological factors (Weiss Score, mitotic count, Ki-67, SF-1 and AVA2, P53, beta-catenin immunohistochemistry, resection status), molecular factors (chromosomal aberrations, methylation profile, altered gene expression and miRNA expression, gene mutations). EVIDENCE SYNTHESIS: The best way to stratify ACC patients and propose the best therapeutic option is to combine clinical, pathological and molecular factors. CONCLUSIONS: Individualizing patients' prognosis and tumor biology appears as a necessary step for personalized medicine. In addition to tumor stage and tumor grade, the genomic classification may precise the risk stratification and thus help defining therapeutic strategy.