Cancers pédiatriques et anomalies du développement : recensement national et analyse de ces associations dans la base Tumeur Et Développement (TED)

Abstract : Background: most of the cancers arising in early childhood result from the embryonic layers and represent, unlike the adults tumors, developmental and implementation accidents of various tissues more than accidents bound to ageing or tissue turnover. Methods: launched in June 2013, the TED (Tumeur Et Développement) study is a national, prospective and retrospective registration of cases combining a pediatric cancer and congenital abnormalities. A data extraction has been performed and the clinical characteristics of these patients are here depicted. Results: from June 2013 to October 2017, 662 patients have been enrolled in the TED database by 27 French medical centres. Data concerning both developmental abnormalities and tumors were available for 566 patients. The most frequent tumors were CNS tumors (19.3%), leukemias (17.8%) and nephroblastomas (15.7%), which were different from that that described by the National Registry of Childhood Cancers. An early cancer diagnoses compared to median age of pediatric cancer occurrence was recorded (4.3 vs 5 years old). Despite the evidence of the already known associations, (type 1 neurofibromatosis and optic glioma, Beckwith-Wiedemann syndrome and nephroblastoma, Down syndrome and leukemia), new interesting associations were found highlighting common embryonic origin (ie rhabdomyosarcoma and musculoskeletal abnormalities), somatic mosaicisms (ie bladder rhabdomyosarcoma and hypospadias). Only 46.3 % of the patients recorded in the database benefited from a genetic counseling. Conclusion: TED is one of the most important and exhaustive national study reporting associations of congenital abnormalities with pediatric cancers. It represents a key collection for describing new mechanisms and genetic pathways involved in tumorigenesis and malformations.
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Master Thesis
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Submitted on : Friday, January 18, 2019 - 4:21:43 PM
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Cyrielle Fouquet. Cancers pédiatriques et anomalies du développement : recensement national et analyse de ces associations dans la base Tumeur Et Développement (TED). Médecine humaine et pathologie. 2018. ⟨dumas-01986279⟩

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