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弥漫性软脑膜胶质瘤病伴多灶性中枢神经系统梗死

Diffuse leptomeningeal gliomatosis associated with multifocal CNS infarcts.

作者信息

Singh M, Corboy J R, Stears J C, Kleinschmidt-DeMasters B K

机构信息

Department of Pathology, University of Colorado Health Sciences Center, Denver 80262, USA.

出版信息

Surg Neurol. 1998 Oct;50(4):356-62; discussion 362. doi: 10.1016/s0090-3019(97)00371-6.

DOI:10.1016/s0090-3019(97)00371-6
PMID:9817460
Abstract

Widespread dissemination of astrocytoma throughout the cerebrospinal fluid is unusual, especially as the initial presentation in a patient, and association with cerebral infarcts is rare. CASE DESCRIPTION A 47-year-old man subacutely developed progressive headache and backache, vomiting, altered consciousness, and numbness in both arms. Brain computerized axial tomography showed calcification in the fourth ventricle and magnetic resonance imaging demonstrated diffuse meningeal enhancement, a lesion in the canal of Magendie, and T2-weighted hyperintense lesions in several locations. Angiography showed vasospasm/vasculopathy involving multiple cerebral vessels. Biopsy of the fourth ventricular mass revealed a subependymoma with an overlying leptomeningeal anaplastic astrocytoma with numerous Rosenthal fibers. The patient was treated with three doses of intrathecal methotrexate via lumbar puncture but suddenly became unresponsive and died 6 days later, 8 weeks after initial symptoms. Autopsy demonstrated the subependymoma and a widespread leptomeningeal anaplastic astrocytoma, which showed no clear-cut origin from the subependymoma, but microscopically infiltrated the left medial temporal lobe. Multiple subacute and acute infarcts of the brain, brain stem, and upper spinal cord were seen only in areas with leptomeningeal tumor. CONCLUSION Glioma cells surrounding and focally permeating central nervous system vessels without lumenal occlusion can lead to vasospasm and widespread infarcts.

摘要

星形细胞瘤在脑脊液中广泛播散并不常见,尤其是作为患者的首发表现,且与脑梗死相关的情况罕见。病例描述:一名47岁男性亚急性起病,出现进行性头痛、背痛、呕吐、意识改变及双臂麻木。脑部计算机断层扫描显示第四脑室钙化,磁共振成像显示弥漫性脑膜强化、马让迪孔区有一病变以及多个部位T2加权高信号病变。血管造影显示累及多条脑血管的血管痉挛/血管病变。第四脑室肿物活检显示为室管膜下瘤,其上覆软脑膜间变性星形细胞瘤伴大量罗森塔尔纤维。患者通过腰椎穿刺接受了3次鞘内甲氨蝶呤治疗,但在初始症状出现8周后的第6天突然无反应并死亡。尸检显示室管膜下瘤及广泛的软脑膜间变性星形细胞瘤,该星形细胞瘤并非明显起源于室管膜下瘤,但显微镜下浸润了左侧颞叶内侧。仅在有软脑膜肿瘤的区域可见脑、脑干及上颈髓的多发亚急性和急性梗死。结论:围绕并局灶性穿透中枢神经系统血管而无管腔闭塞的胶质瘤细胞可导致血管痉挛和广泛梗死。

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