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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 [2]. BWS is characterized by a great variety of clinical features, among which are abdominal wall defects, macroglossia, pre- and post-natal gigantism, earlobe pits or creases, facial nevus flammeus, hypoglycemia, renal abnormalities, and hemihypertrophy. BWS patients have a 7.5% risk of developing (mostly intra-abdominal) childhood tumors. Tumors most frequently found are Wilms tumor(WT), adrenocortical carcinoma (ACC), rhabdomyosarcoma (RMS), and hepatoblastoma (HB). Patients can be classified as having...

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References

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

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

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  3. Alders M, Ryan A, Hodges M et al (2000) Disruption of a novel imprinted Zinc-finger gene, ZNF215, in the Beckwith-Wiedemann syndrome. Am J Hum Genet 66:1473–1484

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

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  5. 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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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. University Professor of Genetics, German Cancer Research Center - DKFZ, Heidelberg, Germany

    Manfred Schwab

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© 2011 Springer-Verlag Berlin Heidelberg

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Mannens, M. (2011). Beckwith-Wiedemann Syndrome Associated Childhood Tumors. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-16483-5_575

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