The VHL gene mutations are associated with the Hypoxia-inducible factor (HIF)/vascular endothelial growth factor (VEGF) signalling pathway, which results in the overexpression of VEGF and platelet-derived growth factor (PDGF) receptors.2,3 This finding leads to the notably angiogenic characteristic of RCC and to the development of targeted therapies for metastasized disease, such as tyrosine kinase inhibitors (TKIs), mammalian target of rapamycin (mTOR) inhibitors and recombinant humanized monoclonal IgG1 antibodies that oppose VEGF.4, , , , , , –11. The gene discussed is VHL; the disease is renal cell adenocarcinoma.