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Pediatric High-Grade Gliomas and DIPG

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Molecular Pathology of Nervous System Tumors

Part of the book series: Molecular Pathology Library ((MPLB,volume 8))

Abstract

Recent genomic studies of pediatric high-grade gliomas (pHGGs) have unraveled important new clues into the pathogenesis of these tumors. One novel insight that was previously unappreciated is the role of epigenetic alterations in pediatric gliomagenesis. This was realized when mutations in histone 3.3/3.1 (H3.3/H3.1) were identified in these tumors. A related concept is that certain genetic alterations only occur in a region-specific manner within the central nervous system. As an example, K27M H3.3/H3.1 mutations are present in high-grade gliomas that arise along the midline of the central nervous system while G34R and G34V H3.3 mutations are present in high-grade gliomas that arise in the cerebral cortex. In addition, the realization that gliomas that harbor histone mutations are mutually exclusive of isocitrate dehydrogenase (IDH) mutant gliomas, which primarily arise in adults, has reinforced the notion that pediatric gliomas and adult gliomas are biologically disparate diseases. Incorporating this new data within the framework of our existing knowledge of the genetic alterations of pHGGs gives new hope that we will successfully identify effective therapies for these tumors in the upcoming decade.

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Author information

Authors and Affiliations

  1. Departments of Pediatrics and Pathology, Preston Robert Tisch Brain Tumor Center, Duke University Medical Center, Durham, NC, USA

    Oren J. Becher M.D.

  2. Division of Hematology-Oncology, Department of Pediatrics, Duke University Medical Center, 450 Research Dr., LSRC Bldg Rm B362, 91001, Durham, NC, 27710, USA

    Kelly L. Barton

  3. Division of Neurological Surgery, Department of Surgery, Duke University Medical Center, 2301 Erwin Rd., Durham, NC, 27705, USA

    Kyle G. Halvorson M.D.

  4. Department of Pathology, Duke University, M265 Davison Bldg, Erwin Rd., DUMC 3712, Durham, NC, 27710, USA

    Roger McLendon M.D.

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  1. Oren J. Becher M.D.
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  2. Kelly L. Barton
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  3. Kyle G. Halvorson M.D.
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  4. Roger McLendon M.D.
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Correspondence to Oren J. Becher M.D. .

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Editors and Affiliations

  1. Associate Professor of Pediatrics and Otolaryngology, NYU Langone Medical Center, New York, New York, USA

    Matthias A. Karajannis

  2. Professor of Pathology and Neurosurgery, NYU Langone Medical Center, New York, New York, USA

    David Zagzag

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Becher, O.J., Barton, K.L., Halvorson, K.G., McLendon, R. (2015). Pediatric High-Grade Gliomas and DIPG. In: Karajannis, M., Zagzag, D. (eds) Molecular Pathology of Nervous System Tumors. Molecular Pathology Library, vol 8. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1830-0_7

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  • DOI: https://doi.org/10.1007/978-1-4939-1830-0_7

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