High expression of vascular endothelial growth factor (VEGF) and its receptor VEGF-R2 in many ACC specimens (9) led to several studies targeting the tumor vasculature in ACC with bevacizumab, an anti-VEGF monoclonal antibody, and sorafenib, a multi-tyrosine kinase inhibitor in combination with paclitaxel which however failed to demonstrate clinical efficacy (10). This evidence concerns the gene VEGFA and adrenal cortex carcinoma.