Yeaney Gabrielle A, O'Connor Siobhan M, Jankowitz Brian T, Hamilton Ronald L
Brain Pathol. 2009 Jan;19(1):167-70. doi: 10.1111/j.1750-3639.2008.00242.x.
A 16-year-old male presented to an emergency room after falling on his head while inebriated. The patient had only a history of recent fatigue and demonstrated no focal neurological deficit. MRI revealed a cystic and solid, enhancing midline cerebellar lesion. A suboccipital craniotomy was performed. Histologically, the mass showed large bizarre cells arranged in sheets with admixed small lymphocytes. The pleomorphic population had ample glassy eosinophilic cytoplasm and intranuclear inclusions. An infiltrating component resembling diffuse astrocytoma could be found in areas. Rosenthal fibers were particularly abundant in the areas of infiltrating glioma. Mitotic activity was very low, and necrosis was absent. Reticulin fibers between individual cells were focally abundant. Glial fibrillary acidic protein and vimentin were strongly expressed in many cells, while synaptophysin and neurofilament protein were not. Ki-67 showed a very low proliferation index. The pathologic diagnosis was pleomorphic xanthoastrocytoma (PXA) of the cerebellum. PXA is a diagnosis typically regarded as a superficial meningocerebral neoplasm. This case is one of sixteen cerebellar PXAs reported in the literature.
一名16岁男性在醉酒后头部着地,被送往急诊室。该患者仅有近期疲劳史,未表现出局灶性神经功能缺损。磁共振成像(MRI)显示小脑中线有一个囊性和实性增强病变。进行了枕下开颅手术。组织学上,肿块显示有大的奇异细胞成片排列,混有小淋巴细胞。多形性细胞群体有丰富的玻璃样嗜酸性细胞质和核内包涵体。在某些区域可发现类似弥漫性星形细胞瘤的浸润成分。在浸润性胶质瘤区域,罗森塔尔纤维特别丰富。有丝分裂活性非常低,无坏死。单个细胞之间的网状纤维局部丰富。许多细胞中胶质纤维酸性蛋白和波形蛋白强烈表达,而突触素和神经丝蛋白不表达。Ki-67显示增殖指数非常低。病理诊断为小脑多形性黄色星形细胞瘤(PXA)。PXA通常被认为是一种浅表性脑膜脑肿瘤。该病例是文献报道的16例小脑PXA之一。