摘要：

目的 报告病理学诊断明确的大脑胶质瘤病和软脑膜胶质瘤病各一例。方法与结果 （1）大脑胶质瘤病：男性患者，48 岁。临床表现为癫痫和中枢神经系统局灶性体征。MRI 显示双侧大脑半球广泛性白质病变，无明显占位效应，增强后病灶无明显强化。肿瘤细胞和增生的神经胶质细胞均表达胶质纤维酸性蛋白（GFAP），Ki-67 抗原标记指数约为10%。（2）软脑膜胶质瘤病：女性患者，30 岁。临床以颅内高压和癫痫发作为主要症状。MRI 显示蛛网膜下隙和脑室进行性扩大，脑沟和脑裂内多发囊性包裹形成，脑实质浸润，增强后脑膜和囊壁病灶明显强化。肿瘤细胞呈密集不均均分布，胞核异型性明显，小血管增生；GFAP 表达阳性，Ki-67 抗原标记指数约为5%。2 例患者组织病理学均符合间变型星形细胞瘤（WHOⅢ级）诊断。以替莫唑胺化疗为治疗原则。结论 颅内胶质瘤病头部MRI 表现具有一定特异性，可提示诊断，应尽早行脑组织活检术以明确诊断。

关键词: 神经胶质瘤, 脑肿瘤, 脑膜肿瘤, 磁共振成像, 免疫组织化学

Abstract:

Objective  To report one case of gliomatosis cerebri (GC) and one case of leptomeningeal gliomatosis (LG), both of them were clearly diagnosed by brain biopsy. Methods and Results  1) The GC patient was a 48-year-old male who presented with epilepsy and focal neurological deficits. Cranial MRI revealed diffuse white matter lesions in bilateral cerebral hemispheres without obvious mass effect and enhancement. Immunohistochemical staining revealed that tumor cells and proliferative glial cells were positive for glial fibrillary acidic protein (GFAP). Ki-67 labeling index was about 10% . 2) The LG patient was a 30-year-old female who mainly presented with high intracranial pressure and epilepsy. Repeated cranial MRI revealed aggressive enlargement of subarachnoid space and ventricles, multiple encapsulated cysts in sulci and fissures, and parenchymal infiltration. Strong enhancement in meninges and the encapsulated cysts could be seen in contrast-enhanced images. Histologically, dense and uneven distribution of tumor cells was the main feature. Obvious atypia of nuclei and proliferation of small vessels could be seen. Tumor cells were positive for GFAP. Ki-67 labeling index was about 5% . By brain biopsy, both cases were diagnosed as anaplastic astrocytoma (WHO Ⅲ). The principle of treatment was temozolomide chemotherapy.  Conclusions  For intracranial gliomatosis, relatively distinct characteristics in brain MRI could indicate the diagnosis. Brain biopsy should be done early as possible for a clear diagnosis.

Key words: Glioma, Brain neoplasms, Meningeal neoplasms, Magnetic resonance imaging, Immunohistochemistry