Familial Wilms' tumour (defined as either bilateral disease or a family history of Wilms' tumour) account for approximately 5% of cases. For those with sporadic (unilateral) disease the risk of Wilms' tumour among their
offspring is low: in a series of 179 children from 96 survivors of unilateral Wilms' (Li, 1988) non had developed the disease (upper 95% CI 2%). Children with WAGR Syndrome, Beckwith-Wiedemann
Syndrome, Denys-Drash syndrome and Perlman Syndrome have an increased risk of Wilms' tumour.
The WT1 gene located at 11p13 was identified in 1989, however, only about a third of patients carry detectable mutations. Thus the development of Wilms' tumour is complex and is likely to involve several other genetic loci. A
number of other genes on chromosome 11p have also been implicated in Wilms' tumour, including the putative WT2 gene (11p15). Loci at 1p, 7p, 16q, 17p, and 19q (the putative FWT2 gene) are also
implicated.
Brown KW and Malik KTA The molecular biology of Wilms’ tumour. (Review) Exp. Rev. Mol. Med. 2001, 14 May. (Free full text, figs and 96 references.)
Coppes MJ, et al. Genetics of Wilms' tumor. Semin Urol Oncol. 1999;17(1):2-10. Review. Related articles (PubMed)
Huff V Wilms tumor genetics. Am J Med Genet 1998 Oct 2;79(4):260-7 Related articles (PubMed)
Pritchard-Jones K Molecular genetic pathways to Wilms tumor. Crit Rev Oncog 1997;8(1):1-27 Related articles (PubMed)
Li FP, et al. Heritable fraction of unilateral Wilms tumor. Pediatrics 1988 Jan;81(1):147-9 Related articles (PubMed)
 Wilms' Tumour Genetics
Wilms' Tumour : Clinical and Epidemiological Information
 Familial Wilms' Tumour
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Hereditary Wilms' tumour (defined as either bilateral disease or a family history of Wilms' tumour) is uncommon. Bonaiti-Pellie and colleagues (1992) analysed family history for 501 Wilms' tumour patients collected by questionnaire and/or interview of
parents. Just 12 patients (2.4%) had a positive family history of Wilms' tumour, while 4.6% had bilateral tumours. Other large series of patients enrolled on clinical trials likewise suggest that the heritable fraction of Wilms' tumour is relatively
small; (Pastore, 1988 and Breslow, 1982). Genes implicated in familial Wilms' tumour include WT1, FWT1 and FWT2. However, further susceptibility genes remain to be identified.
Bonaiti-Pellie C, et al. Genetics and epidemiology of Wilms' tumor: the French Wilms' tumor study. Med Pediatr Oncol 1992;20(4):284-91 Related articles (PubMed)
Pastore G, et al. Epidemiological features of Wilms' tumor: results of studies by the International Society of Paediatric Oncology (SIOP). Med Pediatr Oncol 1988;16(1):7-11 Related articles (PubMed)
Breslow NE, Beckwith JB Epidemiological features of Wilms' tumor: results of the National Wilms' Tumor Study. J Natl Cancer Inst 1982 Mar;68(3):429-36 Related articles (PubMed)
Rapley EA, et al. Evidence for susceptibility genes to familial Wilms tumour in addition to WT1, FWT1 and FWT2. Br J Cancer 2000 Jul;83(2):177-83 Related articles (PubMed)
Medline Search: familial Wilms' tumor (PubMed)
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Wilms' Tumour Genetics
Wilms' Tumour : Clinical and Epidemiological Information
| Wilms' tumor and WAGR syndrome
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Medline Search: Wilms' tumor AND WAGR syndrome (PubMed)
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Medline Search: WAGR Syndrome (PubMed)
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- Related Resources
- WAGR syndrome (Online Mendelian Inheritance in Man, National Center for Biotechnology Information, USA)
- WAGR Syndrome (National Organization for Rare Disorders, Inc.)
| Wilms' Tumour and Beckwith-Wiedemann Syndrome
|
Steenman M, et al. Genetics of Beckwith-Wiedemann syndrome-associated tumors: common genetic pathways. Genes Chromosomes Cancer 2000 May;28(1):1-13 Related articles (PubMed)
Medline Search: Beckwith-Wiedemann syndrome AND Wilms' tumour (PubMed)
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- Beckwith-Wiedemann Syndrome Genetics
- Screening for Wilms' Tumour in Beckwith-Wiedemann
| Denys-Drash Syndrome and Wilms' Tumour
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Denys Drash syndrome is a rare condition characterised by renal failure, pseudohermaphroditism, and Wilms' tumor. It is associated with abnormalities of the WT1 gene on chromosome 11.
Breslow NE, et al. Renal failure in the Denys-Drash and Wilms' tumor-aniridia syndromes. Cancer Res 2000 Aug 1;60(15):4030-2 Related articles (PubMed)
Koziell A, et al. Published erratum appears in Clin Endocrinol (Oxf) 2000 Jun;52(6):801 Frasier syndrome, part of the Denys Drash continuum or simply a WT1 gene associated disorder of intersex and nephropathy? Clin Endocrinol (Oxf) 2000 Apr;52(4):519-24 Related articles (PubMed)
Baird PN, et al. Constitutional mutations in the WT1 gene in patients with Denys-Drash syndrome. Hum Mol Genet 1992 Aug;1(5):301-5 Related articles (PubMed)
Hastie ND Dominant negative mutations in the Wilms tumour (WT1) gene cause Denys-Drash syndrome--proof that a tumour-suppressor gene plays a crucial role in normal genitourinary development. Hum Mol Genet 1992 Aug;1(5):293-5 Related articles (PubMed)
Coppes MJ, et al. Inherited WT1 mutation in Denys-Drash syndrome. Cancer Res 1992 Nov 1;52(21):6125-8 Related articles (PubMed)
Pelletier J, et al. Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome. Cell 1991 Oct 18;67(2):437-47 Related articles (PubMed)
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- Drash Syndrome (National Organization for Rare Disorders, Inc)
- Denys-Drash syndrome (OMIM)
| Perlman Syndrome and Wilms' Tumour
|
Perlman syndrome is a very rare condition characterised by nephromegaly with renal dysplasia and Wilms' tumour, macrosomia, cryptorchidism, and facial anomalies. It was first described by Perlman et al (1973, 1975) and is thought to be an autosomal
recessive trait. When reviewed by Henneveld et al (1999) just 16 children from 9 families had been reported in the literature.
Henneveld HT, et al. Perlman syndrome: four additional cases and review. Am J Med Genet 1999 Oct 29;86(5):439-46 Related articles (PubMed)
Neri G, et al. The Perlman syndrome: familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies. Am J Med Genet 1984 Sep;19(1):195-207 Related articles (PubMed)
Perlman M, et al. Syndrome of fetal gigantism, renal hamartomas, and nephroblastomatosis with Wilms' tumor. Cancer 1975 Apr;35(4):1212-7 Related articles (PubMed)
Perlman M, et al. Renal hamartomas and nephroblastomatosis with fetal gigantism: a familial syndrome. J Pediatr. 1973 Sep;83(3):414-8. Related articles (PubMed)
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Wilms' Tumour Genetics
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| LOH 16q in Wilms' Tumour
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Skotnicka-Klonowicz G, et al. 16q heterozygosity loss in Wilms' tumour in children and its clinical importance. Eur J Surg Oncol 2000 Feb;26(1):61-6 Related articles (PubMed)
Grundy RG, et al. Loss of heterozygosity on chromosome 16 in sporadic Wilms' tumour. Br J Cancer 1998 Nov;78(9):1181-7 Related articles (PubMed)
Grundy PE, et al. Loss of heterozygosity for chromosomes 16q and 1p in Wilms' tumors predicts an adverse outcome. Cancer Res 1994; 54(9):2331-3 Related articles (PubMed)
Mason JE, et al. 16q loss of heterozygosity and microsatellite instability in Wilms' tumor. J Pediatr Surg 2000 Jun;35(6):891-6; discussion 896-7 Related articles (PubMed)
Klamt B, et al. Allele loss in Wilms tumors of chromosome arms 11q, 16q, and 22q correlate with clinicopathological parameters. Genes Chromosomes Cancer 1998 Aug;22(4):287-94 Related articles (PubMed)
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Wilms' Tumour Genetics
Wilms' Tumour : Clinical and Epidemiological Information
Chromosme 16
| LOH 19q in Familial Wilms' Tumour (FWT2 19q13.3-q13.4)
|
McDonald JM, et al. Linkage of familial Wilms' tumor predisposition to chromosome 19 and a two-locus model for the etiology of familial tumors. Cancer Res 1998 Apr 1;58(7):1387-90 Related articles (PubMed)
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Wilms' Tumour Genetics
Wilms' Tumour : Clinical and Epidemiological Information
Chromosme 19
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