Genetic basis for kidney cancer: opportunity for disease-specific approaches to therapy.

National Cancer Institute, Medical Oncology, Urologic Oncology Branch, 10 Center Drive MSC 1107, Building 10 CRC Room 1-5942, Bethesda, Maryland 20892-1107, USA. pfaffee@mail.nih.gov

BACKGROUND: Kidney cancer is not a homogenous entity; it is comprised of many different tumor types, with different biologies and molecular mechanisms leading to disease and therefore different treatment approaches. OBJECTIVE: To describe the genetic basis and biochemical pathways underlying inherited forms of renal cancer, specifically in four described syndromes (von Hippel-Lindau [VHL], hereditary papillary renal cancer [HPRC], Birt-Hogg-Dubé [BHD] and hereditary leiomyomatosis renal cell carcinoma [HLRCC]), and to elucidate how the understanding of these diseases enables the possibility of disease-specific approaches to therapy. METHODS: A systematic review of the published literature on inherited and sporadic forms of renal cancer was performed. CONCLUSION: Understanding of the biology and mechanisms of different forms of kidney cancer provides an opportunity for development of new treatment options.