TY - JOUR
T1 - Diffuse intrinsic pontine glioma
T2 - current insights and future directions
AU - Srikanthan, Dilakshan
AU - Taccone, Michael S.
AU - Van Ommeren, Randy
AU - Ishida, Joji
AU - Krumholtz, Stacey L.
AU - Rutka, James T.
N1 - Funding Information:
The study was supported by grants from the Canadian Institutes of Health Research ( PJT-155967 and PJT-153104), Meagan's Hug (Meagan Bebenek Foundation), b.r.a.i.nchild and the Wiley Fund.
Publisher Copyright:
© 2021, The Author(s).
PY - 2021/12
Y1 - 2021/12
N2 - 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.
AB - 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.
KW - Diffuse intrinsic pontine glioma
KW - Disease models
KW - Molecular genetics
KW - Neuro-oncology
KW - Neurosurgery
KW - Pediatrics
KW - Therapeutics
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U2 - 10.1186/s41016-020-00218-w
DO - 10.1186/s41016-020-00218-w
M3 - Review article
AN - SCOPUS:85099042717
SN - 2057-4967
VL - 7
JO - Chinese Neurosurgical Journal
JF - Chinese Neurosurgical Journal
IS - 1
M1 - 6
ER -
