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Beckwith-Wiedemann Syndrome Associated Childhood Tumors

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Encyclopedia of Cancer

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Definition

Beckwith-Wiedemann syndrome (BWS) is a complex overgrowth disorder caused by a number of genes that are subject to genomic imprinting. A high incidence of solid childhood tumors is seen in patients that present with BWS.

Characteristics

Diagnostic Criteria

Beckwith-Wiedemann syndrome is a disorder first described by Beckwith in 1963 at the 11th annual meeting of the Western Society for Pediatric Research. Later, Wiedemann and Beckwith described the syndrome in more detail (Beckwith 1969). BWS is characterized by a great variety of clinical features, among which are abdominal wall defects, macroglossia, pre- and postnatal gigantism, earlobe pits or creases, facial nevus flammeus, hypoglycemia, renal abnormalities, and hemihypertrophy. BWS patients have a high risk (4.2–25 %, on average 8.6 %) of developing (mostly intra-abdominal) childhood tumors. Tumors most frequently found are Wilms tumor(WT), adrenocortical carcinoma (ACC), rhabdomyosarcoma (RMS), and hepatoblastoma...

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References

  • Beckwith J (1969) Macroglossia, omphalocele, adrenal cytomegaly, gigantism and hyperplastic visceromegaly. Birth Defects 5:188–196

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  • Bliek J, Gicquel C, Maas S et al (2004) Epigenotyping as a tool for the prediction of tumour type and tumour risk in Beckwith-Wiedemann syndrome patients. J Pediatr 145:796–799

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  • Eggermann T, Algar E et al (2014) Clinical utility card for: Beckwith-Wiedemann syndrome. Eur J Hum Genet 22(3). doi:10.1038/ejhg.2013.132. Epub 3 July 2013

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  • Steenman M, Westerveld A, Mannens M (2000) Genetics of Beckwith-Wiedemann syndrome associated tumors: common genetic pathways. Genes Chromosom Cancer 28:1–13

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  • Wiedemann H (1964) Complexe malformatif familial avec hernie ombilicale et macroglossie, un ‘syndrome nouveau’. J Genet Hum 13:223–232

    CAS  PubMed  Google Scholar 

See Also

  • (2012) FOXO1A. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1447. doi:10.1007/978-3-642-16483-5_2256

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  • (2012) P53. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2747. doi:10.1007/978-3-642-16483-5_4331

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Author information

Authors and Affiliations

  1. Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands

    Marcel Mannens

Authors
  1. Marcel Mannens
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Corresponding author

Correspondence to Marcel Mannens .

Editor information

Editors and Affiliations

  1. Abt. Tumorgenetik, Deutsches Krebsforschungszentrum, Heidelberg, Germany

    Manfred Schwab

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Mannens, M. (2015). Beckwith-Wiedemann Syndrome Associated Childhood Tumors. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_575-2

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  • DOI: https://doi.org/10.1007/978-3-642-27841-9_575-2

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Online ISBN: 978-3-642-27841-9

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