New targeted therapies for adrenocortical carcinomas.

Abstract

Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. It has undergone in-depth clinical and laboratory investigations, with the help of the most important research groups all over the world. Nonetheless the cure for this kind of neoplasia is not right around the corner, given its complexity and multi-faceted feature, that lead researchers to think at "one person one ACC." Currently total resection is the most concrete option for ACC patients, whenever possible. Mitotane remains the main drug for primary or adjuvant therapy, but gives partial and unsatisfactory therapeutic results, especially in metastatic ACC. This prompted the researchers to find other ways to fight against this malignancy: targeted therapy seems the most promising answer, as it is based on biomolecular and genetic cancer signature. Numerous specific targets were explored for the treatment of ACC, such as those involving angiogenesis, steroidogenesis, Wnt/β-catenin pathway and many others key factors. Even if large efforts have been made, no effective target therapy entered in the clinical use. This data should not be considered only as detrimental, rather it should propel scientific research to invest more resources into the therapeutic exploration of ACC and in particular on the most promising strategy, the targeted therapy.