世界卫生组织中枢神经系统肿瘤分类的演变:1979-2021年

The evolution of WHO Classification of Tumors of the Central Nervous System from 1979 to 2021

简要回顾世界卫生组织(WHO)中枢神经系统肿瘤分类历史,重点介绍1979年(第一版)至2021年(第五版)WHO中枢神经系统肿瘤分类的演变,包括肿瘤分类框架、类型/亚型和分级。本文讲述第一版至第四版以组织学为基础的分类,历经第四版修订版的尝试以及中枢神经系统肿瘤分类分子信息及实践方法联盟⁃非WHO官方组织的7次更新,发展到2021年WHO中枢神经系统肿瘤分类(简称新版肿瘤分类)中更多的由生物学和分子特征定义的肿瘤实体。儿童型弥漫性胶质瘤由于分子遗传学不同,从弥漫性胶质瘤中独立出来,也是新版肿瘤分类的重要变化之一。本文还以新版肿瘤分类中中枢神经系统肿瘤实体为线索,介绍各个类别肿瘤类型/亚型的演变,包括因何增加、删除或更改命名。新版肿瘤分类中中枢神经系统肿瘤分级方法力求为与非中枢神经系统肿瘤分级相一致,分级标准在组织学特征的基础上增加分子参数。新版肿瘤分类为临床诊疗设定了新标准,期待未来可以促进临床研究和基础科学研究。

In this article,we look back on history of the classification of tumors of central nervous system(CNS)in brief,and further review the evolution of WHO Classification of Tumors of CNS from the first edition to the fifth edition(WHO CNS5),including the category frame,tumor type/subtype and tumor grading.Changed from histology based classification into more biologically and molecularly defined entities,WHO CNS5 was recently released as expected,after attempt in WHO CNS4 updated version and 7 update publications upon the work of the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy⁃Not Official WHO(cIMPACT⁃NOW).One of the important changes in WHO CNS5 is the separating gliomas into pediatric⁃type and adult⁃type based on their well⁃established molecular genetic differences.Tracing alone the clue in WHO CNS5,we introduce evolutionary process of entities and subtypes under each categories,and try to explain why they were added,deleted or why their naming was changed from WHO CNS1 to WHO CNS5.Tumor grading in WHO CNS5 has make efforts on moving close to how grading is done for non⁃CNS neoplasms,and added molecular parameters as biomarkers of grading based on histological features.WHO CNS5 has set new standers for clinical management of CNS tumors,and has been expected to facilitate related clinical studies and basic research in the future.