Pediatric brain tumors comprise a diverse set of diseases. (Epi)genomic analyses have provided insights into the biology of these tumors, stratifying them into distinct subtypes with different oncogenic driver mechanisms and developmental origins. A feature shared by these tumors is their initiation within neural stem or progenitor cells that undergo stalled differentiation in unique, niche-dependent ways. In this review, we provide an overview of how (epi)genomic characterization has revealed pediatric brain tumor origins and underlying biology. We focus on the best characterized tumor types—gliomas, ependymomas, medulloblastomas—as well as select rarer types such as embryonal tumors with multilayered rosettes, atypical teratoid/rhabdoid tumors, and choroid plexus carcinomas in which new insights have been made. The discovery of diverse developmental origins of these tumors and their defining molecular characteristics has led to a better understanding of their etiologies, with important implications for diagnostics, future therapy development, and clinical trial design.Expected final online publication date for the Annual Review of Cancer Biology, Volume 8 is April 2024. Please see http://www.annualreviews.org/page/journal/pubdates for revised estimates.

中文翻译：

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来自儿科脑肿瘤（外延）基因组分析的生物学、诊断和治疗见解

小儿脑肿瘤包括多种疾病。 （表观）基因组分析提供了对这些肿瘤生物学的深入了解，将它们分为具有不同致癌驱动机制和发育起源的不同亚型。这些肿瘤的一个共同特征是它们在神经干细胞或祖细胞中起始，并以独特的、依赖于生态位的方式经历停滞的分化。在这篇综述中，我们概述了（表观）基因组特征如何揭示儿童脑肿瘤的起源和基础生物学。我们专注于最有特征的肿瘤类型——神经胶质瘤、室管膜瘤、髓母细胞瘤——以及一些罕见的类型，如多层玫瑰花结的胚胎肿瘤、非典型畸胎瘤/横纹肌瘤和脉络丛癌，这些肿瘤已取得了新的见解。这些肿瘤的不同发育起源及其定义的分子特征的发现使人们更好地了解其病因，对诊断、未来治疗开发和临床试验设计具有重要意义。癌症年度评论的预计最终在线发布日期《生物学》第 8 卷的发布日期为 2024 年 4 月。请参阅 http://www.annualreviews.org/page/journal/pubdates 了解修订后的估计值。