Abstract
Children with the Beckwith-Wiedemann syndrome have a greatly increased potential for the specific development of the embryonal tumours hepatoblastoma, rhabdomyosarcoma and Wilms' tumour. Data obtained with molecular probes suggest that the association between these disparate, rare tumour types reflects a common pathogenetic mechanism that entails the somatic development of homozygosity for a mutant allele at a locus on human chromosome 11.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Abnormalities, Multiple / genetics*
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Alleles
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Animals
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Carcinoma, Hepatocellular / genetics*
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Child
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Child, Preschool
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Chromosome Mapping
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Chromosomes, Human, 6-12 and X*
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DNA, Neoplasm / analysis
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Female
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Heterozygote
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Humans
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Infant
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Liver Neoplasms / genetics*
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Male
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Mice
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Mutation
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Neoplasm Transplantation
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Nucleic Acid Hybridization
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Osteosarcoma / genetics
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Rhabdomyosarcoma / genetics*
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Sarcoma, Ewing / genetics
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Syndrome