Follow-up studies in the 2000s elucidated the primary oncogenic pathway involved in ccRCC, and the new understanding of the roles of VHL-hypoxia inducible factor (HIF) and vascular endothelial growth factor (VEGF) gave rise to improved treatment options for metastatic ccRCC [1]. The gene discussed is VHL; the disease is nonpapillary renal cell carcinoma.