The link between VHL/HIF pathway alterations and the VEGF axis has been demonstrated by encouraging preliminary activity of pazopanib, a multikinase angiogenesis inhibitor, in von Hippel-Lindau disease associated with RCC; retinal, cerebellar, and spinal haemangioblastomas; pheochromocytomas; pancreatic serous cystadenomas; and pancreatic neuroendocrine tumors [21]. Here, VHL is linked to von Hippel-Lindau disease.