Inherited epithelial tumors of the kidney: old and new diseases

This review summarizes information on inherited epithelial tumors of the ki dney. Emphasis is placed on identifying clinically distinct inherited forms of renal cancer because each distinct clinical syndrome defines a differen t renal cancer susceptibility gene. So far, two genes that predispose to ep ithelial cancers of the kidney have been identified, VHL and the MET proto- oncogene. Available evidence suggests that several renal cancer genes remai n to be identified.