A large-scale genomic analysis of sporadic ccRCC performed by the Cancer Genome Atlas Research Network revealed functional loss of the VHL complex in 90% of ccRCC cases due to mutations in VHL (a tumor suppressor gene), DNA methylation of the VHL promoter, or mutations in TCEB1 encoding elongin C (a component of the VHL complex) [3,4]. Here, ELOC is linked to nonpapillary renal cell carcinoma.