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弥漫性中线胶质瘤的流行病学、诊断策略和治疗进展

英文原题:Epidemiology, Diagnostic Strategies, and Therapeutic Advances in Diffuse Midline Glioma.

PubMed 2023/08/12(内容时间) J Clin Med Q1 · IF 3.3(JCR 2025)

研究概要

目的:弥漫性中线胶质瘤(DMG)是一种高度侵袭性且致命的脑肿瘤,主要影响儿童和年轻成人。

中文摘要

目的:弥漫性中线胶质瘤(DMG)是一种高度侵袭性、致死性脑肿瘤,主要影响儿童和年轻成人。根据 WHO CNS5 命名法,DMG 近期被重新归类并命名为“弥漫性中线胶质瘤”,此前称为弥漫性内生性脑桥胶质瘤(DIPG)或 IV 级脑干胶质瘤;重新分类扩大了 DMG 的患者范围。尽管诊断和治疗已有进展,治疗选择有限仍导致预后不佳。放疗历来是改善患者生存的主要治疗方式。方法:本系统文献综述旨在全面汇总 2012 年 1 月 1 日至 2023 年 7 月 31 日期间 DMG 的诊断和治疗信息。综述遵循 PRISMA(系统综述和荟萃分析优先报告条目)声明,并检索 PubMed、Cochrane Library 和 SciELO 等数据库。结果:目前,DMG 的分子分类对于判断预后和治疗选择日益重要。研究人员依据肿瘤分子组成探索多种新兴治疗途径,包括免疫调节药物、抗 GD2 CAR-T 和抗 GD2 CAR-NK 疗法、提高血脑屏障通透性的技术、异柠檬酸脱氢酶抑制剂、溶瘤疫苗和肽疫苗。然而,仍需更多临床试验以建立可靠的毒性、剂量和疗效指南。结论:H3K27 基因突变的发现推动了部分中线肿瘤重新分类,并扩大了 DMG 患者范围。DMG 研究仍在发展,令人鼓舞的发现凸显高度特异性和个体化治疗策略对于取得成功的重要性。

展开英文摘要原文

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.

论文信息

作者
Miguel Llordes G、Medina Pérez VM、Curto Simón B、Castells-Yus I、Vázquez Sufuentes S、Schuhmacher AJ
单位
Molecular Oncology Group, Instituto de Investigación Sanitaria Aragón (IIS Aragón), 50009 Zaragoza, Spain.Spain
文献类型
综述
期刊
Journal of clinical medicine2023 Aug 12
原文标识
PubMed 37629304 · DOI 10.3390/jcm12165261