High-grade gliomas (HGG) are the most common malignant brain tumors in the pediatric population and account for a large subset of all pediatric central nervous system neoplasms. The management of pediatric HGG continues to be challenging, with poor outcome in many cases despite aggressive treatments. Consequently, parallel research efforts have been focused on identifying the underlying genetic and biological basis of pediatric HGG in order to more clearly define prognostic subgroups for treatment stratification as well as identify new treatment targets. These cutting-edge advances have revolutionized pediatric neuro-oncology and have revealed novel oncogenic vulnerabilities that are being therapeutically leveraged. Promising treatments – including pathway-targeting small molecules as well as epigenetic therapy – are being evaluated in clinical trials, and recent genomic discoveries in rare glioma subgroups have led to the identification of additional new potentially-actionable alterations. This review summarizes the current state of knowledge about the molecular characterization of pediatric HGG in correlation to the revised World Health Organization (WHO) classification, as well as provides an overview of some targeted treatment approaches in the modern clinical management of high-grade gliomas.

高度神经胶质瘤（HGG）是儿科人群中最常见的恶性脑肿瘤，占所有儿科中枢神经系统肿瘤的很大一部分。小儿HGG的治疗仍然具有挑战性，尽管采取了积极的治疗措施，但在许多情况下结果仍然很差。因此，平行的研究工作一直集中在确定小儿HGG的潜在遗传和生物学基础上，以便更清楚地定义治疗分层的预后亚组并确定新的治疗目标。这些最前沿的进展彻底改变了小儿神经肿瘤学，并揭示了正在被治疗利用的新型致癌性漏洞。临床试验中正在评估有前途的治疗方法，包括靶向小分子途径和表观遗传学治疗方法，最近在罕见的神经胶质瘤亚组中发现了基因组，从而发现了新的可能起作用的新变化。这篇综述总结了与修订的世界卫生组织（WHO）分类相关的有关儿科HGG分子表征的知识的现状，并概述了高级神经胶质瘤的现代临床管理中的一些靶向治疗方法。