KDR and adrenal cortex carcinoma: Several inhibitors of VEGFR pathways, such as sorafenib, regorafenib, axitinib and pazopanib, have been studied in ACC and have shown some clinical benefit, with the overall response rate ranging between 0% and 10%, and prolonged stable disease (>6 months) in up to 85% of unselected patients [97,98,99,100].