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Management of Diffuse Pontine Gliomas in Children: Recent Developments

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Abstract

The prognosis for children with diffuse intrinsic pontine gliomas (DIPGs) is dismal. Although DIPGs constitute only 10–15 % of all pediatric brain tumors, they are the main cause of death in this group with a median survival of less than 12 months. Standard therapy involves radiotherapy, which produces transient neurologic improvement. Despite several clinical trials having been conducted, including trials on targeted agents to assess their efficacy, there is no clear improvement in prognosis. However, knowledge of DIPG biology is increasing, mainly as a result of research using biopsy and autopsy samples. In this review, we discuss recent studies in which systemic therapy was administered prior to, concomitantly with, or after radiotherapy. The discussion also includes novel therapeutic options in DIPG. Continuing multimodal and multitargeted therapies might lead to an improvement in the dismal prognosis of the disease.

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Acknowledgments

No sources of funding were used to assist in the preparation of this review. Rejin Kebudi and Fatma Betul Cakir have no conflicts of interest that are directly relevant to the content of this review.

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Authors and Affiliations

  1. Istanbul University Cerrahpasa Medical Faculty Pediatric Hematology-Oncology, P.C: 34090, Millet Street, Capa, Istanbul, Turkey

    Rejin Kebudi

  2. Istanbul University Oncology Institute Pediatric Hematology-Oncology, Istanbul, Turkey

    Rejin Kebudi

  3. Bezmialem Vakif University Medical Faculty Department of Pediatric Hematology-Oncology, Istanbul, Turkey

    Fatma Betul Cakir

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Kebudi, R., Cakir, F.B. Management of Diffuse Pontine Gliomas in Children: Recent Developments. Pediatr Drugs 15, 351–362 (2013). https://doi.org/10.1007/s40272-013-0033-5

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