脑上皮样血管内皮瘤1例及文献复习

Epithelioid hemangioendothelioma of the brain: a case report and literature review

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。

Purpose To study the diagnosis and differential diagnosis of epithelioid hemangiendothelioma of brain. Methods A case of primary multicentric epithelioid hemangiendotholima of the brain was studied by means of HE stain, immunohistochemistry and ultrastructural observation and literature review. Results Tumor usually appeared as small nest or cords composed of eosinophilic epithlioid or spindled cells, supported by prominent fibromyoid stroma. Epithelioid cells often contained cytoplasmic vacuolization. In some vacuoles eryhrocytes could be identified. Ultrastructure study showed characteristic Weibel Palad bodies in cytoplasmic. Immunohistochemical assay showed reactivity for vimentin and endothelial markers FⅧ、CD31、CD34 and UEA 1, but CK、EMA、GFAP、S 100 protein and Mac387 are negative. No expression of AB/PAS; the presence of solid vascular channels can be showed by Ag staining. Conclusion EHE is an uncommon borderline tumor. It occur rarely in an intracranial location. Differential diagnostic considerations of cerebral EHE should include myxoid chondrosarcoma, chordoid meningoma and metastatic carcinomas and atrial myxoma.