Background: Periodic updates to the World Health Organization (WHO) classification system for central nervous system (CNS) tumors reflect advances in the pathological diagnosis, categorization, and molecular underpinnings of primary brain, spinal cord, and peripheral nerve tumors. The 5th edition of the WHO Classification of CNS Tumors was published in 2021. This review discusses the guiding principles of the revision, introduces the more common new diagnostic entities, and describes tumor classification and nomenclature changes that are relevant for pediatric neurological surgeons. Summary: Revisions to the WHO CNS tumor classification system introduced new diagnostic entities, restructured and renamed other entities with particular impact in the diffuse gliomas and CNS embryonal tumors, and expanded the requirements for incorporating both molecular and histological features of CNS tumors into a unified integrated diagnosis. Many of the new diagnostic entities occur at least occasionally in pediatric patients and will thus be encountered by pediatric neurosurgeons. New nomenclature impacts the terminology that is applied in communication between pathologists, surgeons, clinicians, and patients. Requirements for molecular information in tumor diagnosis is expected to refine diagnostic categories while also introducing practical considerations for intra-operative consultation, preliminary histological evaluation, and triaging of neurosurgical tissue samples for histology, molecular testing, and clinical trial requirements. Key Messages: Pediatric brain tumor diagnosis and clinical management is a multidisciplinary effort that is rapidly advancing in the molecular era. Interdisciplinary collaboration is critical for providing the best care for pediatric CNS tumor patients. Pediatric neurosurgeons and their local neuropathologists and neuro-oncologists must work collaboratively to put the most current CNS tumor diagnostic guidelines into standard practice.


中文翻译：

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回顾 WHO 儿童脑和脊髓肿瘤分类的最新变化

背景：世界卫生组织 (WHO) 中枢神经系统 (CNS) 肿瘤分类系统的定期更新反映了原发性脑、脊髓和周围神经肿瘤的病理诊断、分类和分子基础方面的进展。WHO 中枢神经系统肿瘤分类第 5 版于 2021 年出版。这篇综述讨论了修订的指导原则，介绍了更常见的新诊断实体，并描述了与儿科神经外科医生相关的肿瘤分类和命名变化。总结：对 WHO CNS 肿瘤分类系统的修订引入了新的诊断实体，重组并重新命名了对弥漫性神经胶质瘤和 CNS 胚胎性肿瘤具有特殊影响的其他实体，并扩大了将 CNS 肿瘤的分子和组织学特征纳入统一综合诊断的要求。许多新的诊断实体至少偶尔会出现在儿科患者中，因此儿科神经外科医生会遇到。新的命名法影响了病理学家、外科医生、临床医生和患者之间交流中应用的术语。肿瘤诊断中对分子信息的要求有望细化诊断类别，同时还引入了术中会诊、初步组织学评估以及神经外科组织样本分类以用于组织学、分子检测和临床试验要求的实际考虑。关键信息：小儿脑肿瘤的诊断和临床管理是一项在分子时代迅速发展的多学科工作。跨学科合作对于为小儿 CNS 肿瘤患者提供最佳护理至关重要。儿科神经外科医生及其当地的神经病理学家和神经肿瘤学家必须通力合作，将最新的 CNS 肿瘤诊断指南纳入标准实践。