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


CONCLUSIONS
Understanding of the biology and mechanisms of different forms of kidney cancer provides an opportunity for development of new treatment options.
Pfaffenroth
Elizabeth Cartwright
EC
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
Linehan
W Marston
WM
D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D002292:Carcinoma, Renal Cell / Q000235:genetics / Q000628:therapy; D015195:Drug Design; D005649:Fumarate Hydratase / Q000172:deficiency / Q000235:genetics / Q000502:physiology; D016147:Genes, Tumor Suppressor; D020022:Genetic Predisposition to Disease; D006801:Humans; D007680:Kidney Neoplasms / Q000235:genetics* / Q000628:therapy; D018231:Leiomyomatosis / Q000235:genetics / Q000628:therapy; D009363:Neoplasm Proteins / Q000037:antagonists & inhibitors / Q000235:genetics / Q000502:physiology; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000628:therapy; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics* / Q000628:therapy; D011518:Proto-Oncogene Proteins / Q000037:antagonists & inhibitors / Q000172:deficiency / Q000235:genetics / Q000502:physiology; D019859:Proto-Oncogene Proteins c-met; D011519:Proto-Oncogenes; D017978:Receptors, Growth Factor / Q000037:antagonists & inhibitors / Q000172:deficiency / Q000235:genetics / Q000502:physiology; D025521:Tumor Suppressor Proteins / Q000172:deficiency / Q000235:genetics / Q000502:physiology; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics / Q000502:physiology; D006623:von Hippel-Lindau Disease / Q000235:genetics / Q000628:therapy
D000970:Antineoplastic Agents; C476078:FLCN protein, human; D009363:Neoplasm Proteins; D011518:Proto-Oncogene Proteins; D017978:Receptors, Growth Factor; D025521:Tumor Suppressor Proteins; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D051794:Von Hippel-Lindau Tumor Suppressor Protein; C491743:MET protein, human; D019859:Proto-Oncogene Proteins c-met; D005649:Fumarate Hydratase; C497446:VHL protein, human