目标：弥漫性中线胶质瘤（DMG）是一种高度侵袭性和致命性的脑肿瘤，主要影响儿童和年轻成年人。根据WHO CNS5命名法，DMG最近被重新分类为“弥漫性中线胶质瘤”，扩大了DMG的人群范围，以前被称为弥漫性内部桥脑胶质瘤（DIPG）或第四级脑干胶质瘤。尽管在诊断和治疗方面取得了进展，但有限的治疗选择导致预后不佳。放射治疗在提高患者生存率方面一直是主要的治疗方法。方法：本系统文献综述旨在全面汇编2012年1月1日至2023年7月31日期间关于DMG的诊断和治疗信息。本综述遵循PRISMA（系统综述和Meta分析的首选报告项目）声明，并利用PubMed、Cochrane图书馆和SciELO等数据库。结果：目前，DMG的分子分类在确定预后和治疗选择方面的作用日益重要。基于肿瘤的分子组成，正在探索新兴的治疗途径，包括免疫调节剂、抗GD2 CAR T细胞和抗GD2 CAR-NK疗法、增加血脑屏障通透性的技术、异柠檬酸脱氢酶抑制剂、溶瘤疫苗和多肽疫苗。然而，还需要更多的临床试验来建立毒性、剂量和疗效的可靠指南。结论：H3K27基因突变的发现导致某些中线肿瘤的重新分类，扩大了DMG的人群范围。DMG研究领域仍在不断发展，以实现治疗成功，高度特异性和量身定制的治疗策略的重要性得到了进一步强调。

Object: Diffuse midline glioma (DMG) is a highly aggressive and lethal brain tumor predominantly affecting children and young adults. Previously known as diffuse intrinsic pontine glioma (DIPG) or grade IV brain stem glioma, DMG has recently been reclassified as "diffuse midline glioma" according to the WHO CNS5 nomenclature, expanding the DMG demographic. Limited therapeutic options result in a poor prognosis, despite advances in diagnosis and treatment. Radiotherapy has historically been the primary treatment modality to improve patient survival. Methods: This systematic literature review aims to comprehensively compile information on the diagnosis and treatment of DMG from 1 January 2012 to 31 July 2023. The review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement and utilized databases such as PubMed, Cochrane Library, and SciELO. Results: Currently, molecular classification of DMG plays an increasingly vital role in determining prognosis and treatment options. Emerging therapeutic avenues, including immunomodulatory agents, anti-GD2 CAR T-cell and anti-GD2 CAR-NK therapies, techniques to increase blood-brain barrier permeability, isocitrate dehydrogenase inhibitors, oncolytic and peptide vaccines, are being explored based on the tumor's molecular composition. However, more clinical trials are required to establish solid guidelines for toxicity, dosage, and efficacy. Conclusions: The identification of the H3K27 genetic mutation has led to the reclassification of certain midline tumors, expanding the DMG demographic. The field of DMG research continues to evolve, with encouraging findings that underscore the importance of highly specific and tailored therapeutic strategies to achieve therapeutic success.