Von Hippel–Lindau (VHL) syndrome is associated with pheochromocytomas, paragangliomas and pancreatic neoplasia, and is caused by the loss of the VHL tumour suppressor gene, regulating the hypoxia-inducible factor (HIF) and vascular endothelial growth factor (VEGF) pathways [31,32,33]. The gene discussed is VEGFA; the disease is von Hippel-Lindau disease.