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Management of diffuse intrinsic pontine glioma in children: current and future strategies for improving prognosis

    Erica C Kaye

    Department of Oncology, St Jude Children's Research Hospital; 262 Danny Thomas Place, Mail Stop 260, Memphis, TN 38105, USA

    Department of Pediatrics, University of Tennessee Health Sciences Center, TN, USA

    ,
    Justin N Baker

    Department of Oncology, St Jude Children's Research Hospital; 262 Danny Thomas Place, Mail Stop 260, Memphis, TN 38105, USA

    Department of Pediatrics, University of Tennessee Health Sciences Center, TN, USA

    &
    Alberto Broniscer

    *Author for correspondence:

    E-mail Address: alberto.broniscer@stjude.org

    Department of Oncology, St Jude Children's Research Hospital; 262 Danny Thomas Place, Mail Stop 260, Memphis, TN 38105, USA

    Department of Pediatrics, University of Tennessee Health Sciences Center, TN, USA

    Published Online:https://doi.org/10.2217/cns.14.47

    SUMMARY 

    Diffuse intrinsic pontine glioma (DIPG) is one of the deadliest pediatric central nervous system cancers in spite of treatment with radiation therapy, the current standard of care. The outcome of affected children remains dismal despite multiple clinical trials that investigated radiation therapy combined with chemotherapy. Recently, multiple genome-wide studies unveiled the distinct molecular characteristics of DIPGs and preclinical models of DIPG were developed to mimic the human disease. Both of these accomplishments have generated tremendous progress in the research of new therapies for children with DIPG. Here we review some of these promising new strategies.

    Papers of special note have been highlighted as: • of interest; •• of considerable interest

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