Abstract
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.
Highlights
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
Clear cell is the most common type (75%), papillary occurs in 10%, and chromophobe renal carcinoma occurs in 5%, with the remaining being made up of collecting duct, medullary, and oncocytoma
The von Hippel Lindau (VHL) gene has the characteristics of a tumor suppressor gene, and alteration of both copies of the gene is found in VHL-associated tumors and sporadic clear cell renal carcinoma (Fig. 2)
Summary
Identification of the Genes for Kidney Cancer: Opportunity for Disease-SpecificTargeted Therapeutics. Pinto,1Ramaprasad Srinivasan,1Maria Merino, Peter Choyke, Lynda Choyke,1Jonathan Coleman,1Jorge Toro, Gladys Glenn, CathyVocke,1Bert Zbar, Laura S.
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