VHL and nonpapillary renal cell carcinoma: Patients with germline mutations of VHL are at risk of developing ccRCC with up to 600 tumors and 1100 cysts per kidney [5]; somatic mutations leading to biallellic inactivation of VHL occur in 50%–60% of sporadic ccRCCs [6]; VHL promoter methylation was observed in 15% of ccRCCs [7].