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2016年世界卫生组织中枢神经系统肿瘤分类概述

摘要

The 2016 World Health Organization classiifcation of tumors of the central nervous system is both a conceptual and practical advance over its 2007 predecessor. For the ifrst time, the WHO classiifcation of CNS tumors uses molecular parameters in addition to histology to deifne many tumor entities, thus formulating a concept for how CNS tumor diagnoses should be structured in the molecular era. As such, the 2016 CNS WHO presents major restructuring of the diffuse gliomas, medulloblastomas and other embryonal tumors, and incorporates new entities that are defined by both histology and molecular features, including glioblastoma, IDH-wildtype and glioblastoma, IDH-mutant; diffuse midline glioma, H3 K27M–mutant; RELA fusion–positive ependymoma; medulloblastoma, WNT-activated and medulloblastoma, SHH-activated;and embryonal tumor with multilayered rosettes, C19MC-altered. The 2016 edition has added newly recognized neoplasms, and has deleted some entities, variants and patterns that no longer have diagnostic and/or biological relevance. Other notable changes include the addition of brain invasion as a criterion for atypical meningioma and the introduction of a soft tissue-type grading system for the now combined entity of solitary ifbrous tumor/hemangiopericytoma-a departure from the manner by which other CNS tumors are graded. Overall, it is hoped that the 2016 CNS WHO will facilitate clinical, experimental and epidemiological studies that will lead to improvements in the lives of patients with brain tumors.%2016版世界卫生组织(World Health Organization,WHO)中枢神经系统(central nervous system,CNS)肿瘤分类相对于2007版是一个概念和实践上的进步,首次在组织学的基础上使用分子学的特征来进行肿瘤分类,从而为分子时代CNS肿瘤诊断构建了一个新的概念。2016版CNS WHO分类主要是对弥漫性胶质瘤、髓母细胞瘤和其他胚胎性肿瘤进行了重新分类,纳入了用组织学和分子学特点定义的一些新的分类,包括胶质母细胞瘤IDH-野生型和胶质母细胞瘤IDH-突变型;弥漫型中线胶质瘤H3 K27M–突变型;室管膜瘤RELA融合-阳性;髓母细胞瘤-WNT激活型和髓母细胞瘤-SHH激活型;多层菊形团样胚胎性肿瘤C19MC-改变。2016版新增加了最近公认的肿瘤,同时删除了一些不具有诊断和/或生物关联性的种类、变异型和形式。另外一些显著的变化包括将对脑组织的侵袭性作为不典型脑膜瘤的一个诊断标准,以及为孤立性纤维肿瘤/血管上皮肿瘤联合体引入了与其他CNS肿瘤的分级方式不同的软组织类型分级系统。总之,2016年CNS WHO分类会使临床、实验及流行病学的研究更加便利,并且改善脑肿瘤患者的生存。

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