Natsumeda Manabu
Department of Neurosurgery, Brain Research Institute, Niigata University.
No Shinkei Geka. 2023 Sep;51(5):876-883. doi: 10.11477/mf.1436204829.
Pediatric-type diffuse high-grade glioma is a new tumor class defined in the 5 edition of the WHO Classification of Tumors of the Central Nervous System(WHO2021). The class includes the following four tumor types: diffuse midline glioma, H3 K27-altered; diffuse hemispheric glioma, H3 G34-mutant; diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype; and infant-type hemispheric glioma. Genetic detection of histone H3 mutations as well as detection of fusion genes and even methylation classifiers may be necessary to diagnose these tumors. Therefore, understanding their clinical and radiographical features is vital. Urgent establishment of new treatments is necessary for diffuse midline glioma, H3 K27-altered, as it is associated with very poor prognosis and is generally resistant to temozolomide. In this chapter, we focus on the clinical, radiographical, pathological, and molecular features of pediatric-type diffuse high-grade gliomas and introduce promising new treatments for diffuse midline glioma, H3 K27-altered.
儿童型弥漫性高级别胶质瘤是《世界卫生组织中枢神经系统肿瘤分类》第5版（WHO2021）中定义的一种新的肿瘤类型。该类型包括以下四种肿瘤：弥漫性中线胶质瘤，H3 K27改变型；弥漫性半球胶质瘤，H3 G34突变型；弥漫性儿童型高级别胶质瘤，H3野生型且异柠檬酸脱氢酶（IDH）野生型；以及婴儿型半球胶质瘤。诊断这些肿瘤可能需要检测组蛋白H3突变、融合基因甚至甲基化分类器。因此，了解它们的临床和影像学特征至关重要。对于弥漫性中线胶质瘤，H3 K27改变型，迫切需要建立新的治疗方法，因为其预后非常差，且通常对替莫唑胺耐药。在本章中，我们重点关注儿童型弥漫性高级别胶质瘤的临床、影像学、病理和分子特征，并介绍针对弥漫性中线胶质瘤，H3 K27改变型的有前景的新治疗方法。
