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Identification of the Genes for Kidney Cancer: Opportunity for Disease-Specific Targeted Therapeutics

Authors' Affiliations: ¹ Urologic Oncology Branch, ² Laboratory of Pathology, and ³ Molecular Imaging Program, Center for Cancer Research and Genetic Epidemiology Branch; ⁴ Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, Maryland; and ⁵ Basic Research Program, Science Applications International Corporation-Frederick, Inc., National Cancer Institute-Frederick, Frederick, Maryland

Requests for reprints: W. Marston Linehan, Urologic Oncology Branch, National Cancer Institute, Room 1-5940, Building 10, Bethesda, MD 20892-1107. Phone: 301-496-6353; Fax: 301-402-0922; E-mail: wml{at}nih.gov.

Recent advances in understanding the kidney cancer gene pathways has provided the foundation for the development of targeted therapeutic approaches for patients with this disease. Kidney cancer is not a single disease; it includes a number of different types of renal cancers, each with different histologic features, a different clinical course, a different response to therapy, and different genes causing the defects. Most of what is known about the genetic basis of kidney cancer has been learned from study of the inherited forms of kidney cancer: von Hippel Lindau (VHL gene), hereditary papillary renal carcinoma (c-Met gene), Birt Hogg Dubé (BHD gene), and hereditary leiomyomatosis renal cell cancer (fumarate hydratase gene). These Mendelian single-gene syndromes provide a unique opportunity to evaluate the effectiveness of agents that target the VHL, c-Met, BHD, and fumarate hydratase pathways.