Inherited epithelial tumors of the kidney: old and new diseases.

ABSTRACT

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