Tumor and Development (TED)
Condition: Tumor and Abnormalities of the Development
MeSH terms: Congenital Abnormalities
Study type: Observational

Summary: 
      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.
    

Detailed description: 
      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
    

Intervention name(s): blood and tumor samples
Intervention type(s): Other
Intervention description(s): all tumor pathology associated with anomaly of development
Observational model: Cohort
Time perspective: Other
Sampling method: Non-Probability Sample
Study population: 
        Patient having developed a cancerous pathology and presenting one or several anomalies of
        the development.
      
Keywords: Pediatric patients, tumor, developmental abnormalities