Clinicopathology of diffuse intrinsic pontine glioma and its redefined genomic and epigenomic landscape

Eshini Panditharatna; Kurt Yaeger; Lindsay B Kilburn; Roger J Packer; Javad Nazarian

doi:10.1016/j.cancergen.2015.04.008

Clinicopathology of diffuse intrinsic pontine glioma and its redefined genomic and epigenomic landscape

Cancer Genet. 2015 Jul-Aug;208(7-8):367-73. doi: 10.1016/j.cancergen.2015.04.008. Epub 2015 May 1.

Authors

Eshini Panditharatna¹, Kurt Yaeger², Lindsay B Kilburn³, Roger J Packer⁴, Javad Nazarian⁵

Affiliations

¹ Institute for Biomedical Sciences, George Washington University School of Medicine, Washington, DC, USA; Research Center for Genetic Medicine, Children's National Health System, Washington, DC, USA.
² Department of Neurosurgery, Georgetown University School of Medicine, Washington, DC, USA.
³ Division of Oncology, Center for Cancer and Immunology Research, Children's National Health System, Washington, DC, USA.
⁴ Brain Tumor Institute, Center for Neuroscience and Behavioral Medicine, Children's National Health System, Washington, DC, USA.
⁵ Research Center for Genetic Medicine, Children's National Health System, Washington, DC, USA; Department of Integrative Systems Biology, George Washington University School of Medicine and Health Sciences, Washington, DC, USA. Electronic address: jnazarian@childrensnational.org.

PMID: 26206682
DOI: 10.1016/j.cancergen.2015.04.008

Abstract

Diffuse intrinsic pontine glioma (DIPG) is one of the most lethal pediatric central nervous system (CNS) cancers. Recently, a surge in molecular studies of DIPG has occurred, in large part due to the increased availability of tumor tissue through donation of post-mortem specimens. These new discoveries have established DIPGs as biologically distinct from adult gliomas, harboring unique genomic aberrations. Mutations in histone encoding genes are shown to be associated with >70% of DIPG cases. However, the exact molecular mechanisms of the tumorigenicity of these mutations remain elusive. Understanding the driving mutations and genomic landscape of DIPGs can now guide the development of targeted therapies for this incurable childhood cancer.

Keywords: DIPG; High grade glioma; brainstem glioma; diffuse intrinsic pontine glioma; glioma.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Brain Stem Neoplasms / genetics*
Brain Stem Neoplasms / pathology
Brain Stem Neoplasms / therapy
Child
Epigenomics*
Genetic Heterogeneity
Genetic Predisposition to Disease / genetics*
Genomics*
Glioma / genetics*
Glioma / pathology
Glioma / therapy
Histones / genetics
Humans
Mutation*

Substances

Histones