Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, Nebraska, USA.
Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri, USA.
Indian J Pathol Microbiol. 2022 May;65(Supplement):S24-S32. doi: 10.4103/ijpm.ijpm_1109_21.
Glioblastoma is the most common malignant central nervous system (CNS) tumor in adults. Acute common clinical symptoms include headache, seizure, behavior changes, focal neurological deficits, and signs of increased intracranial pressure. The classic MRI finding of glioblastoma is an irregularly shaped, rim-enhancing or ring-enhancing lesion with a central dark area of necrosis. This constellation of features correlates with microscopic findings of tumor necrosis and microvascular proliferation. Besides these common features, several well-recognized histological subtypes include giant cell glioblastoma, granular cell glioblastoma, gliosarcoma, glioblastoma with a primitive neuronal component, small cell glioblastoma, and epithelioid glioblastoma. While glioblastoma was historically classified as isocitrate dehydrogenase (IDH)-wildtype and IDH-mutant groups, the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) and the fifth edition of the WHO Classification of Tumors of the Central Nervous System clearly updated the nomenclature to reflect glioblastoma to be compatible with wildtype IDH status only. Therefore, glioblastoma is now defined as "a diffuse, astrocytic glioma that is IDH-wildtype and H3-wildtype and has one or more of the following histological or genetic features: microvascular proliferation, necrosis, Telomerase reverse transcriptase promoter mutation, Epidermal growth factor receptor gene amplification, +7/-10 chromosome copy-number changes (CNS WHO grade 4)."
胶质母细胞瘤是成人中最常见的恶性中枢神经系统 (CNS) 肿瘤。急性常见的临床症状包括头痛、癫痫、行为改变、局灶性神经功能缺损和颅内压升高的迹象。胶质母细胞瘤的典型 MRI 表现为形状不规则、边缘增强或环形增强病变，伴有中央坏死的暗区。这种特征组合与肿瘤坏死和微血管增殖的显微镜发现相关。除了这些常见特征外，还有几种公认的组织学亚型，包括巨细胞胶质母细胞瘤、颗粒细胞胶质母细胞瘤、胶质肉瘤、具有原始神经元成分的胶质母细胞瘤、小细胞胶质母细胞瘤和上皮样胶质母细胞瘤。虽然胶质母细胞瘤历史上被分类为异柠檬酸脱氢酶（IDH）-野生型和 IDH-突变型，但中枢神经系统肿瘤分类的信息分子和实用方法联盟（cIMPACT-NOW）和第五版世界卫生组织中枢神经系统肿瘤分类明确更新了命名法，以反映胶质母细胞瘤仅与野生型 IDH 状态兼容。因此，胶质母细胞瘤现在被定义为“弥漫性星形细胞瘤，IDH 野生型和 H3 野生型，具有以下一种或多种组织学或遗传特征：微血管增殖、坏死、端粒酶逆转录酶启动子突变、表皮生长因子受体基因扩增、+7/-10 染色体拷贝数变化（CNS WHO 分级 4）”。
