Diffuse midline gliomas (DMGs) are a group of aggressive CNS tumors, primarily affecting children and young adults, which have historically been associated with dismal outcomes. As the name implies, they arise in midline structures in the CNS, primarily in the thalamus, brainstem, and spinal cord. In more recent years, significant advances have been made in our understanding of DMGs, including molecular features, with the identification of potential therapeutic targets. We aim to provide an overview of the most recent updates in the field of DMGs, including classification, molecular subtypes, diagnostic techniques, and emerging therapeutic strategies including a review of the ongoing clinical trials, thus providing the treating multidisciplinary team with a comprehensive understanding of the current landscape and potential therapeutic strategies for this devastating group of tumors.
弥漫性中线胶质瘤(DMGs)是一类侵袭性中枢神经系统肿瘤,主要影响儿童和青少年,其预后历来极差。顾名思义,这类肿瘤起源于中枢神经系统的中线结构,主要位于丘脑、脑干和脊髓。近年来,随着对DMGs分子特征的深入认识及潜在治疗靶点的发现,该领域取得了显著进展。本文旨在综述DMGs领域的最新进展,涵盖分类体系、分子亚型、诊断技术以及新兴治疗策略(包括对当前临床试验的评述),从而为临床多学科诊疗团队提供关于这类难治性肿瘤的现状认知与潜在治疗策略的全面参考。
Decoding Diffuse Midline Gliomas: A Comprehensive Review of Pathogenesis, Diagnosis and Treatment