儿童低级别胶质瘤（pLGGs）是幼儿最常见的脑肿瘤。尽管通常与良好的整体生存率相关，这些中枢神经系统肿瘤的儿童经常经历慢性肿瘤和治疗相关的病态。此外，患有不可切除肿瘤的个体往往出现多次复发和持续的神经系统症状。对pLGGs的深度分子分析表明，它们是由在单一有丝分裂原激活通路（MEK/ERK）上汇聚的遗传改变引起的，但它们的生长受其局部微环境中非肿瘤细胞（神经元、T细胞、小胶质细胞）的严重影响。肿瘤细胞MEK/ERK通路激活与间质细胞支持之间的相互作用需要使用预测性临床前模型来筛选最有前景的药物候选物以进行临床评估。作为一系列关注pLGGs的白皮书的一部分，我们讨论了目前的临床前pLGG建模现状，旨在改善对这些常见脑肿瘤儿童的临床转化。© 作者 2023. 版权由牛津大学出版社代表神经肿瘤学会保留。如需授权，请发送电子邮件至：journals.permissions@oup.com。

Pediatric low-grade gliomas (pLGGs) are the most common brain tumor in young children. While they are typically associated with good overall survival, children with these central nervous system tumors often experience chronic tumor- and therapy-related morbidities. Moreover, individuals with unresectable tumors frequently have multiple recurrences and persistent neurological symptoms. Deep molecular analyses of pLGGs reveal that they are caused by genetic alterations that converge on a single mitogenic pathway (MEK/ERK), but their growth is heavily influenced by nonneoplastic cells (neurons, T cells, microglia) in their local microenvironment. The interplay between neoplastic cell MEK/ERK pathway activation and stromal cell support necessitates the use of predictive preclinical models to identify the most promising drug candidates for clinical evaluation. As part of a series of white papers focused on pLGGs, we discuss the current status of preclinical pLGG modeling, with the goal of improving clinical translation for children with these common brain tumors.© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.