Introduction: Embryonal brain tumors (EBTs) are highly aggressive malignancies predominantly affecting children. They include medulloblastoma (MB), atypical rhabdoid/teratoid tumors (ATRT), pineoblastoma (PB), embryonal tumor multiple rosettes (ETMR)/C19MC-altered tumors, and newly recognized embryonal tumors with FOXR2 activation or BCOR alteration.Areas covered: This review will provide a comprehensive overview and updated of the literature on each of these EBTs. The evolution from location- and histopathology-based diagnosis to more specific and robust molecular-based classification schemes, as well as treatment modalities, will be discussed.Expert commentary: The subgrouping of EBTs with multi-omic profiling has had important implications for risk stratification and discovery of targetable driver pathways. However, these innovations are unlikely to significantly improve survival among high-risk patients until robust preclinical studies are conducted, followed by validation in biology-informed clinical trials.

中文翻译：

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分子时代的小儿胚胎脑肿瘤。

简介：胚胎脑瘤（EBTs）是高度侵袭性的恶性肿瘤，主要影响儿童。它们包括髓母细胞瘤（MB），非典型横纹肌/类畸形肿瘤（ATRT），成胚细胞瘤（PB），胚胎肿瘤多花丛（ETMR）/ C19MC改变的肿瘤以及新发现的具有FOXR2激活或BCOR改变的胚胎肿瘤。审查将提供有关这些EBT的文献的全面概述和最新资料。将讨论从基于位置和组织病理学的诊断到更具体，更可靠的基于分子的分类方案以及治疗方式的演变。专家评论：具有多组学分析的EBT分组对风险分层具有重要意义以及发现目标驱动程序路径。然而，