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• W2077755570 abstract "Les syndromes de prédisposition aux cancers de l’enfant semblent bien connu ; parmi eux, certains sont associés à une pathologie plus large du développement : syndromes de Wiedeman-Beckwith, neurofibromatose de type 1, défaut de réparation de l’ADN, syndrome de Drash et WAGR… De nombreuses associations de malformations et de cancer dans l’enfance restent inexpliquées et la question se pose alors du caractère fortuit ou bien significatif de ces associations. Le protocole « TED » (tumeur et développement) a pour objectif de recenser toutes ces associations afin d’en identifier les récurrences et de permettre d’orienter les recherches sur des causes génétiques aux cancers. Cette étude multicentrique utilise la base CEMARA (centre des maladies rares) déjà existante afin de limiter les risques de redondance et de permettre une connexion aisée, tant avec les bases de données des généticiens qu’avec les registres de cancers de l’enfant. Cette étude devrait permettre une description plus méthodique des syndromes malformatifs associés aux cancers et ainsi identifier de nouveaux syndromes de prédisposition. Predisposition syndromes in childhood cancers seem well known; among them some are associated with a broader pathology of development: Beckwith-Wiedeman syndrome, neurofibromatosis type 1, DNA repair defects Drash syndrome WAGR… However, many associations of malformations and cancer in childhood remain unexplained, and the question arises of incidental or significance of these associations. The protocol “TED” (Tumor and Development) aims at identifying all these associations, studying their recurrence and helping more basic research on the genetic causes of pediatric cancer. This multicenter study uses the database CEMARA (Centre for Rare Diseases) already existing in order to limit the risk of redundancy and allows easy connection with both geneticists’ databases and with the cancer registries. This study should allow a more systematic description of malformation syndromes associated with cancer and lead to discover new predisposition syndromes." @default.
• W2077755570 created "2016-06-24" @default.
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• W2077755570 date "2014-06-01" @default.
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• W2077755570 title "Tumeur et développement (TED) : un enregistrement national des associations de cancers pédiatriques et de pathologies malformatives" @default.
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• W2077755570 doi "https://doi.org/10.1016/j.oncohp.2013.10.008" @default.
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