最常见的儿童中枢神经系统（CNS）肿瘤是小儿低级别胶质瘤（pLGG），占儿童所有中枢神经系统肿瘤的30%-40%。尽管相关发病率很高，但与肿瘤相关的死亡率相对较少。 pLGG 现在被概念化为一种慢性疾病，强调了功能结果和生活质量指标的重要性。关于这些肿瘤的大量数据已经出现，包括更好地了解它们的自然史和分子驱动因素，为使用靶向抑制剂铺平了道路。虽然这些治疗方法预示着巨大的前景，但如何最好地优化其使用仍然存在挑战，并且与这些抑制剂相关的长期毒性仍然未知。国际儿科低级别胶质瘤联盟 (iPLGGc) 是一个由拥有 pLGG 专业知识的医生和科学家组成的全球团体，致力于解决关键的 pLGG 问题。在这里，iPLGGc 概述了 pLGG 的当前最新技术，包括流行病学、组织学、分子景观、治疗范式、生存结果、功能结果、成像反应和持续的挑战。本文还介绍了其他 3 篇 pLGG 手稿，内容涉及 (1) pLGG 临床前模型，(2) 在 pLGG 中进行早期临床试验的共识框架，以及 (3) pLGG 耐药、反弹和复发。© 作者(s) 2023。由牛津大学出版社代表神经肿瘤学会出版。版权所有。如需权限，请发送电子邮件至：journals.permissions@oup.com。

The most common childhood central nervous system (CNS) tumor is pediatric low-grade glioma (pLGG), representing 30%-40% of all CNS tumors in children. Although there is high associated morbidity, tumor-related mortality is relatively rare. pLGG is now conceptualized as a chronic disease, underscoring the importance of functional outcomes and quality-of-life measures. A wealth of data has emerged about these tumors, including a better understanding of their natural history and their molecular drivers, paving the way for the use of targeted inhibitors. While these treatments have heralded tremendous promise, challenges remain about how to best optimize their use, and the long-term toxicities associated with these inhibitors remain unknown. The International Pediatric Low-Grade Glioma Coalition (iPLGGc) is a global group of physicians and scientists with expertise in pLGG focused on addressing key pLGG issues. Here, the iPLGGc provides an overview of the current state-of-the-art in pLGG, including epidemiology, histology, molecular landscape, treatment paradigms, survival outcomes, functional outcomes, imaging response, and ongoing challenges. This paper also serves as an introduction to 3 other pLGG manuscripts on (1) pLGG preclinical models, (2) consensus framework for conducting early-phase clinical trials in pLGG, and (3) pLGG resistance, rebound, and recurrence.© 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.