Diffuse intrinsic pontine glioma: current insights and future directions

Dilakshan Srikanthan, Michael S. Taccone, Randy Van Ommeren, Joji Ishida, Stacey L. Krumholtz, James T. Rutka

Research output: Contribution to journalReview articlepeer-review

2 Citations (Scopus)

Abstract

Diffuse intrinsic pontine glioma (DIPG) is a lethal pediatric brain tumor and the leading cause of brain tumor–related death in children. As several clinical trials over the past few decades have led to no significant improvements in outcome, the current standard of care remains fractionated focal radiation. Due to the recent increase in stereotactic biopsies, tumor tissue availabilities have enabled our advancement of the genomic and molecular characterization of this lethal cancer. Several groups have identified key histone gene mutations, genetic drivers, and methylation changes in DIPG, providing us with new insights into DIPG tumorigenesis. Subsequently, there has been increased development of in vitro and in vivo models of DIPG which have the capacity to unveil novel therapies and strategies for drug delivery. This review outlines the clinical characteristics, genetic landscape, models, and current treatments and hopes to shed light on novel therapeutic avenues and challenges that remain.

Original languageEnglish
Article number6
JournalChinese Neurosurgical Journal
Volume7
Issue number1
DOIs
Publication statusPublished - Dec 2021
Externally publishedYes

Keywords

  • Diffuse intrinsic pontine glioma
  • Disease models
  • Molecular genetics
  • Neuro-oncology
  • Neurosurgery
  • Pediatrics
  • Therapeutics

ASJC Scopus subject areas

  • Surgery
  • Neurology
  • Clinical Neurology

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