Glioblastoma with primitive neuronal component, a rare neoplasm, is recognized as a distinct histological pattern of glioblastoma. In this study we report the morphological and immunohistochemical features of three cases of glioblastoma with primitive neuronal component diagnosed at the Institute along with a comprehensive literature review. The cases include: (1) 11-year-old girl with right fronto-parietal lesion, (2) 48-year-old male with right parietal lesion, and (3) 36-year-old male with left fronto-parietal lesion. Case 1 had prior history of glioblastoma. All the cases had classic morphology of glioblastoma along with GFAP-negative and synaptophysin-positive primitive neuronal component. The latter was poorly demarcated from the glial component in case 1, while well-defined in the remaining two. All the three cases exhibited diffuse p53 positivity and a higher MIB-1 labelling index in the neuronal component compared to the glial component. One of them (case 3) was IDH1 R132H-mutant with loss of ATRX expression. None were positive for K27M-mutant H3 or G34R-mutant H3.3. Literature review of 50 published cases of glioblastoma with primitive neuronal component was performed. The age of onset ranged from 3 months to 82 years (mean: 50 years) with M:F of 1.5:1. 18.8% of tumors were IDH-mutant, 87.5% were p53 positive and three cases showed H3F3A gene mutations. There was a greater propensity for neuraxial dissemination, noted in 20% of cases. Overall survival of glioblastoma with primitive neuronal component was similar to that of IDH-wildtype glioblastoma (13 months) which was significantly shorter compared to the overall survival of IDH-mutant glioblastoma (33.6 months).

具有原始神经元成分的胶质母细胞瘤是一种罕见的肿瘤，被认为是胶质母细胞瘤的独特组织学模式。在这项研究中，我们报告了在研究所诊断出的三例具有原始神经元成分的胶质母细胞瘤的形态学和免疫组织化学特征，并进行了全面的文献综述。病例包括：（1）11岁女孩右额顶叶病变，（2）48岁男性右顶叶病变，（3）36岁男性左额顶叶病变. 病例 1 有胶质母细胞瘤病史。所有病例均具有典型的胶质母细胞瘤形态以及 GFAP 阴性和突触素阳性的原始神经元成分。后者与病例 1 中的神经胶质成分界限不清，而在其余两个中则明确界定。与神经胶质成分相比，所有三个病例在神经元成分中均表现出弥漫性 p53 阳性和更高的 MIB-1 标记指数。其中之一（案例 3）是 IDH1 R132H 突变体，ATRX 表达缺失。K27M 突变体 H3 或 G34R 突变体 H3.3 均未呈阳性。对 50 例已发表的具有原始神经元成分的胶质母细胞瘤病例进​​行了文献回顾。发病年龄为 3 个月至 82 岁（平均：50 岁），M:F 为 1.5:1。18.8% 的肿瘤为 IDH 突变，87.5% 为 p53 阳性，3 例显示 H3F3A 基因突变。在 20% 的病例中注意到椎管内扩散的倾向更大。