Diffuse intrinsic pontine glioma:current insights and future directions  

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

机构地区：[1]Cell Biology Program,The Hospital for Sick Children,686 Bay St,Toronto,ON M5G 0A4,Canada [2]The Arthur and Sonia Labatt Brain Tumour Research Centre,The Hospital for Sick Children,686 Bay St,Toronto,ON M5G 0A4,Canada [3]Department of Laboratory Medicine and Pathobiology,University of Toronto,Toronto,ON,Canada [4]Division of Neurosurgery,Department of Surgery,The Ottawa Hospital,Ottawa,ON,Canada [5]Developmental and Stem Cell Biology Program,The Hospital for Sick Children,686 Bay St,Toronto,ON M5G 0A4,Canada [6]Institute of Medical Sciences,University of Toronto,Toronto,ON,Canada [7]Division of Neurosurgery,Department of Surgery,The Hospital for Sick Children,Suite 1503,555,University Avenue,Toronto,ON M5G 1X8,Canada

出　　处：《Chinese Neurosurgical Journal》2021年第4期289-299,共11页中华神经外科杂志（英文）

基　　金：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.

摘　　要：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.

关 键 词：Diffuse intrinsic pontine glioma Molecular genetics NEURO-ONCOLOGY THERAPEUTICS Disease models PEDIATRICS NEUROSURGERY 

分 类 号：R739.41[医药卫生—肿瘤]