摘要： 目的   探讨中枢神经系统胶质母细胞瘤的临床表现及病理学特征。方法   回顾分析1 例伴印戒细胞样细胞和神经节细胞分化的中枢神经系统胶质母细胞瘤患者的临床表现、组织病理学和免疫表型特点，采用免疫组织化学染色方法诊断与鉴别诊断，并复习相关文献。结果   男性患者，29 岁。临床主要表现为头晕、头痛、记忆力减退并渐进性加重，伴双眼视力下降。MRI 检查右侧额叶近中线灰白质区占位性病变伴出血。手术中可见肿瘤瘤体主要位于右侧额叶，小部分沿胼胝体浸润至左侧额叶，大小约为4.50 cm × 5.00 cm × 5.00 cm，与正常脑组织边界不清；肿瘤呈实性、灰红色、质地柔软、血液供应丰富。光学显微镜观察肿瘤主要由胶质分化的细胞组成；肿瘤细胞排列呈实性片状，散在单核或多核瘤巨细胞，可见周围有肿瘤细胞呈假“栅栏”样排列的小灶性坏死及呈肾小球样的间质血管增生；肿瘤细胞具有明显的异型性、细胞核深染以及核分裂象；部分肿瘤细胞呈印戒细胞样细胞和神经节细胞分化特征。免疫组织化学染色肿瘤细胞胞质胶质纤维酸性蛋白、神经微丝蛋白、巢蛋白和蛋白基因产物9.5 表达阳性，部分肿瘤细胞胞质嗜铬素A 和突触素表达阳性，肿瘤细胞胞核P53 蛋白表达阳性，Ki-67 抗原标记指数（MIB-1）为20% ~ 30%。结论   中枢神经系统胶质母细胞瘤是恶性程度最高的星形细胞肿瘤，也是中枢神经系统好发的肿瘤，但伴印戒细胞样细胞和神经节细胞分化的胶质母细胞瘤临床少见，好发于成年人，预后不良。

关键词: 胶质母细胞瘤, 病理学, 星形细胞瘤, 免疫组织化学

Abstract: Objective To study the clinical manifestations and pathological features of glioblastoma of central nervous system (CNS). Methods The clinical manifestations, histopathology and immunophenotype features were studied in one case of glioblastoma of CNS with signet-ring-like cell and ganglion cell differentiation, immunohistochemistrical assay was used for diagnosis and differentiation, and related literatures were reviewed. Results A 29-year-old man mainly presented with dizziness, headache, and progressive aggravated descent memory and vision. MRI demonstrated a mass associated with hemorrhage, involving gray-white matter of the right frontal lobe near the median line. The tumor measuring 4.50 cm × 5.00 cm × 5.00 cm had abundant blood supply and part of it invaded the left frontal lobe along the corpus callosum. The mass was solid, soft and grey-red in color. The histopathological examination revealed the tumor mainly composed of gliomatous cells arranging in solid sheets. Multi- or mono-nucleated giant cells, vascular proliferation and palisading necrosis were observed. The tumor cells were characterized by atypia, hyperchromasia and high mitotic activity, and some cells presented as signet-ring-like cell and ganglion cell differentiation. The immunohistochemistry showed that glial fibrillary acidic protein (GFAP), neurofilament protein (NF), nestin (Nes) and protein gene product 9.5 (PGP9.5) were positive in cytoplasm, and some cells expressed chromogranin A (CgA) and synaptophysin (Syn), and P53 protein (P53) in nuclei of tumor cells was positive. Ki-67 nuclear antigen index (MIB-1) was 20%-30%. Conclusion Glioblastoma is the most malignant astrocytoma and common primary tumor of CNS, but rarely with signet-ring-like cell and ganglion cell differentiation. It usually occurs in adults between 45 and 70 years old. The prognosis is poor.

Key words: Glioblastoma, Pathology, Astrocytoma, Immunohistochemistry