PMID- 11826016 OWN - NLM STAT- MEDLINE DCOM- 20020312 LR - 20190503 IS - 1468-6244 (Electronic) IS - 0022-2593 (Print) IS - 0022-2593 (Linking) VI - 39 IP - 1 DP - 2002 Jan TI - Familial adult renal neoplasia. PG - 1-5 AB - Our understanding of the molecular mechanisms underlying the tumorigenesis of renal cell carcinoma (RCC) has partially come from studies of RCC related familial cancer syndromes such as von Hippel-Lindau (VHL) disease and hereditary papillary RCC (HPRC). These studies have led to the identification of RCC related genes, which, besides allowing accurate diagnosis of these diseases, have been found mutated or abnormally expressed in the sporadic counterparts of these familial renal tumours. To date, a number of renal tumour related syndromes have been described. We review recent advances in this field and discuss a genetic approach to managing familial cases of renal tumours occasionally encountered by cancer geneticists and urologists. FAU - Takahashi, M AU - Takahashi M AD - Laboratory of Cancer Genetics, Van Andel Research Institute, Grand Rapids, MI 49503, USA. FAU - Kahnoski, R AU - Kahnoski R FAU - Gross, D AU - Gross D FAU - Nicol, D AU - Nicol D FAU - Teh, B T AU - Teh BT LA - eng PT - Journal Article PT - Review PL - England TA - J Med Genet JT - Journal of medical genetics JID - 2985087R SB - IM MH - Adult MH - *Carcinoma, Papillary/genetics/pathology MH - *Carcinoma, Renal Cell/genetics/pathology MH - Genotype MH - Humans MH - *Kidney Neoplasms/genetics/pathology MH - Phenotype MH - *von Hippel-Lindau Disease/genetics/pathology PMC - PMC1734964 EDAT- 2002/02/05 10:00 MHDA- 2002/03/13 10:01 PMCR- 2005/01/01 CRDT- 2002/02/05 10:00 PHST- 2002/02/05 10:00 [pubmed] PHST- 2002/03/13 10:01 [medline] PHST- 2002/02/05 10:00 [entrez] PHST- 2005/01/01 00:00 [pmc-release] AID - 10.1136/jmg.39.1.1 [doi] PST - ppublish SO - J Med Genet. 2002 Jan;39(1):1-5. doi: 10.1136/jmg.39.1.1.