Identification of the genes for kidney cancer: opportunity for disease-specific targeted therapeutics.
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.
Linehan
W Marston
WM
Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA. wml@nih.gov
Pinto
Peter A
PA
Srinivasan
Ramaprasad
R
Merino
Maria
M
Choyke
Peter
P
Choyke
Lynda
L
Coleman
Jonathan
J
Toro
Jorge
J
Glenn
Gladys
G
Vocke
Cathy
C
Zbar
Bert
B
Schmidt
Laura S
LS
Bottaro
Donald
D
Neckers
Len
L
D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics*; D008297:Male; D008954:Models, Biological; D008957:Models, Genetic; D009154:Mutation; D010597:Pharmacogenetics; D025521:Tumor Suppressor Proteins / Q000378:metabolism; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics
D025521:Tumor Suppressor Proteins; D051794:Von Hippel-Lindau Tumor Suppressor Protein; C497446:VHL protein, human