Given the shared pVHL–HIF pathway tumor biology implicated in both VHL-associated disease and sporadic RCC, its functional consequence of inappropriate angiogenesis, and the proven anti-tumor efficacy and regulatory approvals of VEGFR TKI therapies in sporadic advanced clear cell RCC, these antiangiogenic agents were among the first tested for the systemic treatment of patients with VHL disease-associated RCC and other neoplasms (Table 1). The gene discussed is KDR; the disease is neoplasm.