Familial and hereditary renal cancer syndromes.
Hereditary and familial forms of kidney cancer are encountered routinely in urologic practice. Discoveries in the genetic and molecular biology of these diseases have had a critical impact on the understanding of kidney cancer pathogenesis in nearly all subtypes of renal cortical neoplasms and their clinical features. Developing knowledge in the field has helped formulate new diagnostic and molecular therapeutic strategies for patients who have kidney cancer. This article aims to familiarize the reader with the current understanding of identified syndromes, their biology, and approaches to treatment.
Coleman
Jonathan A
JA
Department of Surgery/Urology Division, Weill Cornell Medical College, Memorial Sloan Kettering Cancer Center, Box 12, 1275 York Avenue, NY 10021, USA. colemaj1@mskcc.org
D000231:Adenocarcinoma, Papillary / Q000175:diagnosis / Q000235:genetics; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000235:genetics*; D018231:Leiomyomatosis / Q000175:diagnosis / Q000235:genetics; D009154:Mutation; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis / Q000235:genetics*; D012871:Skin Diseases / Q000175:diagnosis / Q000235:genetics; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000235:genetics; D006623:von Hippel-Lindau Disease / Q000175:diagnosis / Q000235:genetics*