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Relevance of Fusion Genes in Pediatric Cancers: Toward Precision Medicine.


ABSTRACT: Pediatric cancers differ from adult tumors, especially by their very low mutational rate. Therefore, their etiology could be explained in part by other oncogenic mechanisms such as chromosomal rearrangements, supporting the possible implication of fusion genes in the development of pediatric cancers. Fusion genes result from chromosomal rearrangements leading to the juxtaposition of two genes. Consequently, an abnormal activation of one or both genes is observed. The detection of fusion genes has generated great interest in basic cancer research and in the clinical setting, since these genes can lead to better comprehension of the biological mechanisms of tumorigenesis and they can also be used as therapeutic targets and diagnostic or prognostic biomarkers. In this review, we discuss the molecular mechanisms of fusion genes and their particularities in pediatric cancers, as well as their relevance in murine models and in the clinical setting. We also point out the difficulties encountered in the discovery of fusion genes. Finally, we discuss future perspectives and priorities for finding new innovative therapies in childhood cancer.

SUBMITTER: Dupain C 

PROVIDER: S-EPMC5363511 | biostudies-literature | 2017 Mar

REPOSITORIES: biostudies-literature

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Relevance of Fusion Genes in Pediatric Cancers: Toward Precision Medicine.

Dupain Célia C   Harttrampf Anne Catherine AC   Urbinati Giorgia G   Geoerger Birgit B   Massaad-Massade Liliane L  

Molecular therapy. Nucleic acids 20170209


Pediatric cancers differ from adult tumors, especially by their very low mutational rate. Therefore, their etiology could be explained in part by other oncogenic mechanisms such as chromosomal rearrangements, supporting the possible implication of fusion genes in the development of pediatric cancers. Fusion genes result from chromosomal rearrangements leading to the juxtaposition of two genes. Consequently, an abnormal activation of one or both genes is observed. The detection of fusion genes ha  ...[more]

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