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Review
. 2014 Nov;3(6):421-31.
doi: 10.2217/cns.14.47.

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

Erica C Kaye  1 , Justin N Baker, Alberto Broniscer
Affiliations
Review

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

Erica C Kaye et al. CNS Oncol. 2014 Nov.

Abstract

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.

Keywords: glioma; immunotherapy; pontine; prognosis; targeted; therapy.

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Conflict of interest statement

Financial & competing interests disclosure

A Broniscer received partial financial support from Genentech, OSI Pharmaceuticals, AstraZeneca, Takeda Pharmaceuticals, and GlaxoSmithKline to conduct clinical trials. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Figures

<b>Figure 1.</b>
Figure 1.. Typical and atypical MRI features of diffuse intrinsic pontine glioma.
(A) Provides an example of typical MRI findings seen in DIPG. Patient A was diagnosed at the age of 13.3 years with a 2-week history, was treated with radiation therapy and investigational targeted agents, and survived 7 months. Glioblastoma (WHO grade 4) was confirmed at autopsy. (B & C) demonstrate atypical MRI features characterized by tumors with better delineated margins. Patient B was diagnosed at the age of 3.3 years after a 1-week history. Tumor biopsy showed a primitive neuroectodermal tumor. This patient remains alive more than 3 years after intensive therapy. Patient C was diagnosed at the age of 8.8 years with a 2-week history. Tumor biopsy confirmed a fibrillary astrocytoma (WHO grade 2). This patient was treated with radiation therapy and investigational targeted agents and remains alive and well 31 months after diagnosis.
<b>Figure 2.</b>
Figure 2.. Prevalence of specific mutations and copy number abnormalities seen in diffuse intrinsic pontine glioma.
CNA: Copy number abnormality.
<b>Figure 3.</b>
Figure 3.. In an optimal model, children with diffuse intrinsic pontine glioma and their families receive care from a primary neuro-oncology team, with early integration of pediatric palliative care that begins at the time of diagnosis and continues throughout the illness trajectory in cooperation with the primary medical team.
See this image and copyright information in PMC

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