In ACC, angiogenesis is thought to play a key role in tumor growth and survival, as evidenced by the influence of vasculogenic mimicry in tumor growth, overexpression of VEGFR on the surface of tumor cells, and elevated serum levels of VEGF in patients with ACC.18,19 Several completed and 2 ongoing trials have sought to assess the efficacy of VEGFR TKIs in treating unresectable or metastatic ACC. This evidence concerns the gene VEGFA and adrenal cortex carcinoma.