Diffuse intrinsic pontine glioma: current insights and future directions

• 作者: Srikanthan Dilakshan Taccone Michael S. Ommeren Randy Van Ishida Joji Krumholtz Stacey L. Rutka James T.
• 来源期刊: 中华神经外科杂志（英文）
• 关键词: Diffuse intrinsic pontine glioma Molecular genetics Neuro-oncology Therapeutics Disease models Pediatrics Neurosurgery
• 年，卷（期）: 2021,（1）
• 所属期刊栏目: Review
• 页码范围: 68-78
• 页数: 11页
• 语种: 中文
• DOI: 10.1186/s41016-020-00218-w

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.