
Clear cell kidney cancer. Von Hippel-Lindau (VHL) is an autosomal hereditary cancer disorder in which affected individuals are at risk for the development of bilateral, multifocal kidney cancer (A,B) of clear cell histologic type (C). VHL is characterized by germline mutation of the VHL gene (D). The VHL gene is mutated in 57%–89% of sporadic, noninherited clear cell kidney cancers (Gnarra et al. 1994; Moore et al. 2011). (Fig. 2 from Linehan et al. 2003.)











