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新近公布的WHO(2016)CNS肿瘤分类第4版的补充版(4+1),打破了完全基于显微镜下诊断的百年诊断原则,将分子学改变加入CNS肿瘤分类中,开启了形态学+分子病理诊断模式,以及整合诊断的新概念,即组织学分类、分级+分子遗传学改变的诊断模式。另外新分类删除了部分肿瘤名称,如纤维型、原浆型星形细胞瘤、细胞型室管膜瘤,新版认为这些肿瘤仅是一种形态模式,而非独立病种。取消了大脑胶质瘤病及原始神经外胚层肿瘤(PNET)的名称,主要认为大脑胶质瘤病包含不同的基因和表观遗传学亚型,而这些亚型与传统分类的胶质瘤分子学亚型相对应,可根据IDH及1p/19q表型进一步细分,而无需采用大脑胶质瘤病名称。同理PNET也存在着不同的基因和表观遗传学亚型,可根据基因细分或由胚胎性肿瘤替代。新分类另一显著的特点是新增了部分病理类型,主要有上皮性胶质母细胞瘤;弥漫中线胶质瘤伴H3 K27M突变;伴RELA融合基因阳性的室管膜瘤;间变型多形性黄瘤性星形细胞瘤;弥漫性软脑膜胶质神经元肿瘤;大脑多结节、囊泡状节细胞瘤等,本文对部分少见新增肿瘤做一简介。
The newly published WHO (2016) Supplement (4 + 1) of the fourth edition of the CNS Oncology Category 4 (4 + 1) breaks the century-old diagnostic principle of complete microscopic diagnosis by incorporating molecular changes into the CNS oncology classification, opening up morphological + molecular Pathological diagnosis mode, and the new concept of integrated diagnosis, that is, histological classification, classification + molecular genetic changes in the diagnostic mode. In addition, the new classification deleted some of the tumor names, such as fibroids, papillary astrocytomas, and cytotrophoblastomas. The new version considers these tumors as only a morphological pattern, not an independent disease. Canceled the names of brain gliomatosis and primitive neuroectodermal tumors (PNET), which mainly consist of different gene and epigenetic subtypes, which are associated with the traditional classification of glioma molecules Subtypes corresponding to the study can be subdivided according to the IDH and 1p / 19q phenotypes without the need to use the name of gliomatosis. Similarly, there are different PNET gene and epigenetic subtypes, according to gene subdivision or by embryonic tumors instead. Another significant feature of the new classification is the addition of some pathological types, mainly epithelial glioblastoma; diffuse midline glioma with H3 K27M mutation; associated with RELA fusion gene-positive ependymoma; anaplastic polymorphism Sexual xanthomatous astrocytoma; diffuse pia mater glial neuronal tumor; multiple nodules of the brain, vesicular ganglioneurocytoma, etc. In this article, a brief introduction of some rare new tumors.