Srikanthan Dilakshan, Taccone Michael S, Van Ommeren Randy, Ishida Joji, Krumholtz Stacey L, Rutka James T
Cell Biology Program, The Hospital for Sick Children, 686 Bay St, Toronto, ON, M5G 0A4, Canada.
The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, 686 Bay St, Toronto, ON, M5G 0A4, Canada.
Chin Neurosurg J. 2021 Jan 11;7(1):6. doi: 10.1186/s41016-020-00218-w.
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.
弥漫性脑桥内生型胶质瘤（DIPG）是一种致命的儿童脑肿瘤，也是儿童脑肿瘤相关死亡的主要原因。在过去几十年里，多项临床试验并未带来显著的疗效改善，目前的标准治疗方法仍然是分次局部放疗。由于最近立体定向活检的增加，肿瘤组织的可得性推动了我们对这种致命癌症的基因组和分子特征的研究进展。多个研究小组已经在DIPG中鉴定出关键的组蛋白基因突变、基因驱动因素和甲基化变化，为我们了解DIPG的肿瘤发生提供了新的见解。随后，DIPG的体外和体内模型得到了更多的开发，这些模型有能力揭示新的治疗方法和药物递送策略。本综述概述了DIPG的临床特征、基因概况、模型以及当前的治疗方法，希望能阐明仍然存在的新治疗途径和挑战。
