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目的 :探讨脑上皮样血管内皮瘤 (EHE)的诊断及鉴别诊断。方法 :通过HE、免疫组化及电镜观察 1例脑原发性多灶性上皮样血管内皮瘤、并复习文献。结果 :肿瘤由排列呈巢状、索状的上皮样细胞组成 ,胞浆丰富、嗜酸性 ,内含明显空泡呈“印戒”状 ,有的空泡内可见红血球 ,间质含粘液样基质。免疫组化染色显示FⅧ、CD34、CD31、UEA 1和vimentin阳性 ;而CK、CEA、EMA、GFAP、S 10 0蛋白及Mac 387阴性 ;组织化学染色AB/PAS阴性 ,网状纤维显示无数血管腔隙结构。电镜观察胞质内可见特征性Weibel Palade小体。结论 :EHE是一种不常见的生物学行为介于良、恶性之间的血管肿瘤 ,发生在脑内很罕见。组织形态需与转移癌、粘液性软骨肉瘤、脊索样脑膜瘤及转移性心房粘液瘤等鉴别
Objective: To investigate the diagnosis and differential diagnosis of brain epithelioid hemangioendothelioma (EHE). Methods: One case of brain primary multifocal hemangioendothelioma was observed by HE, immunohistochemistry and electron microscopy. The literature was reviewed. Results: The tumor consisted of epithelial cells arranged in nests and cords. The cytoplasm was rich in cytoplasm and eosinophilic. The obvious vacuoles were in the form of “signet ring”. Some vacuoles showed erythrocytes, interstitial mucoid matrix . Immunohistochemical staining showed that FⅧ, CD34, CD31, UEA 1 and vimentin were positive, while CK, CEA, EMA, GFAP, S 10 0 and Mac 387 were negative; histochemical staining was negative for AB / PAS; Gap structure. Electron microscopy showed characteristic Weibel Palade bodies in the cytoplasm. CONCLUSIONS: EHE is an uncommon vascular tumor of biological behavior between benign and malignant and rare in the brain. Tissue morphology and metastasis, mucinous chondrosarcoma, chordate-like meningioma and metastatic atrial myxoma differential