Department of Pathology, Tata Memorial Hospital and ACTREC, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India.
Indian J Pathol Microbiol. 2022 May;65(Supplement):S50-S58. doi: 10.4103/ijpm.ijpm_1202_21.
Pediatric-type of diffuse high-grade gliomas (HGG) are classified as a distinct group in the current fifth edition of WHO classification. This group of high-grade tumors is no more called as glioblastoma (GBM), which has been reserved for adult isocitrate dehydrogenase (IDH)-wild type HGG. These tumors are uncommon as compared to embryonal tumors and low-grade gliomas (LGG). Pediatric-type of diffuse HGG biologically differs from their adult counterparts in that they are therapeutically less sensitive to alkylating chemotherapies. They comprise a heterogeneous group of molecularly defined tumors - predominantly histone gene altered, less common receptor tyrosine kinase (RTK)-mediated, and syndrome-associated. This review provides an overview of these uncommon tumors and discusses the diagnostic approach of this heterogeneous group of tumors.
儿科型弥漫性高级别神经胶质瘤（HGG）在现行的世界卫生组织（WHO）分类的第五版中被归类为一个独特的组别。这类高级别肿瘤不再被称为胶质母细胞瘤（GBM），GBM 已被保留用于成人异柠檬酸脱氢酶（IDH）野生型 HGG。与胚胎性肿瘤和低级别胶质瘤（LGG）相比，儿科型弥漫性 HGG 较为少见。儿科型弥漫性 HGG 在生物学上与成人弥漫性 HGG 不同，它们对烷化化疗药物的敏感性较低。它们由一组分子定义明确的肿瘤组成，主要是组蛋白基因改变，较少见的受体酪氨酸激酶（RTK）介导，以及与综合征相关。本综述介绍了这些罕见肿瘤，并讨论了这组异质性肿瘤的诊断方法。
