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目的探讨脑膜血管瘤病(MA)的临床、影像学和组织学特点及鉴别诊断。方法报告脑膜血管瘤病伴少突胶质细胞瘤1例并复习文献。结果散发性MA发病较年轻,临床表现以癫多见;影像学差异较大,无特异性。本例镜下大部分呈MA的组织学特点,即软脑膜上皮细胞、脑膜血管和血管周梭形纤维母细胞结节状或旋涡状增生,形成边界清楚的皮质内斑块,另见少许少突胶质细胞瘤成分(WHOⅡ级)。免疫组化:梭形细胞vimentin和CD34(+),EMA和CKAE1/AE3(-)。结论 MA是罕见的良性病变,可伴发少突胶质细胞瘤、脑膜瘤等;单纯切除是首选治疗,预后良好。
Objective To investigate the clinical, imaging and histological characteristics and differential diagnosis of meningioma (MA). Methods Report of 1 case of medulloblastoma with oligodendroglioma and review of the literature. Results The incidence of sporadic MA was younger, the clinical manifestations of epilepsy more common; imaging differences, non-specific. In this case, most of the histological features of MA were histological features of pial epithelial cells, meninges and perivascular fusiform fibroblasts nodular or spiral-shaped hyperplasia, the formation of a clear border within the plaque, see also a little less Promyelocytoma component (WHO grade II). Immunohistochemistry: spindle cells vimentin and CD34 (+), EMA and CKAE1 / AE3 (-). Conclusion MA is a rare benign disease, which may be associated with oligodendroglioma and meningiomas. Simple resection is the first choice and the prognosis is good.