Molecular genetics of renal cell carcinoma.

Renal cell carcinoma can be divided into sporadic and inheritable forms. One of the most common inheritable forms occurs in von Hippel-Lindau disease (VHL). VHL is a multisystem neoplastic disorder in which individuals develop tumors and cysts in multiple organs including the kidneys. Careful linkage analysis and cloning identified the VHL gene on chromosome 3p. In accordance with Knudson's hypothesis and tumor suppression theory, the VHL gene has been shown to have an important role in sporadic renal carcinoma as well. Further understanding of the VHL protein is likely to enhance our understanding of renal carcinogenesis, and clinical implications are beginning to evolve.