Tumor and Development (TED)

Overview

The overall project goal is to build a database of childhood cancers associated with developmental anomalies; it aims at identifying new syndromes of genetic predisposition and at enabling the further study of their molecular basis.

Most of the solid cancers arising in the childhood develop from embryonic tissues. The frequent association of paediatric cancers and abnormalities of the development underlines the link between oncogenesis and embryogenesis. However, beside the known malformative syndromes predisposing to one or several types of tumours with a variable penetrance (NF1, Wiedemann-Beckwith, Denys-Drash, Fanconi disease), associations between abnormalities of the development and tumours are badly known and little investigated, and are not listed at present systematically in the registers of child cancers. The cytogenetic exploration of malformative syndromes associated to tumours historically allowed to describe constitutional chromosomal abnormalities of major interest for the understanding of oncogenesis pathways of the most frequent sporadic tumours (del 11p13 and WT1; del 13q14 and Rb1). So, a rare and even exceptional clinical presentation can enrich the knowledge of a common pathology. Our objective is to analyze in a detailed and multidisciplinary way the largest number of possible cases of unusual presentation associating pediatric Tumor And abnormality of Development (TAD). Principle objective * Registration of developmental abnormalities in pediatric patients with cancer retrospectively and prospectively for a period of three years on a nationwide scale Secondary objectives * to record tumoral pathologies in known contexts of cancer predisposition, * to record tumoral pathologies occurring in association with one or more developmental anomaly, these associations might have been already described or not * to identify and locate the biological samples of patients registered in coordination with the national pediatric biobank project * to characterize the molecular basis of the identified associations between developmental abnormalities and tumors. These molecular studies are not straight included in the present project specifically, but should be further conducted on the basis of the clinical data and thanks to the biobank network. * a biannual analysis of aggregated data by a steering committee will be done to identify informative associations that warrant further clinical studies and biological data * Biological studies will be performed in conjunction with local investigators and officials of the local biobank, and in coordination with the operation of BIOCAP

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Outcomes