Genetic and clinical aspects of familial renal neoplasms

Genetic studies of families at high risk for developing malignant and benign renal neoplasms led to cloning of genes whose alteration results in tumor formation. These genes are either tumor suppressors (VHL, TSC) or oncogenes (MET). Their significance in understanding oncogenesis extends far beyond the familial syndromes. The identification of these genes and the elucidation of their biochemical functions are likely to facilitate (1) our understanding of the full clinical spectrum of the corresponding diseases, (2) genetic counseling, and (3) rational design of effective strategies to prevent and/or treat familial and sporadic forms of these neoplasms.