【摘要】 目的 :探讨上皮样血管内皮瘤的临床病理学特征。方法 :对 6例上皮样血管内皮瘤进行HE、组化及免疫组化 (S P法 )检测 ,1例作电镜观察。结果 :年龄 2 6～ 6 6岁 ,平均 38岁。部位 :头颈部 4例 ,上肢、会阴各 1例。形态学特征 :瘤细胞似上皮细胞样 ,呈巢状索状管腔样排列 ,瘤细胞内有原始血管腔 ,核分裂象少见 ,个别病例见细胞有异型性及小灶性坏死 ,间质多少不一黏液样变或透明变。AB/PAS(pH 1 0 )染色 5例 ,瘤细胞阴性 ,AB黏液样基质呈淡蓝色。网染显示大量血管腔隙。免疫组化检测 ,vimentin、CD346例均阳性 ,FⅧRAg、CK 4例阳性 ,CEA、EMA、MAC3876例均阴性。电镜观察 1例见胞质内含有W P小体。结论 :上皮样血管内皮瘤属于低度恶性软组织肿瘤 ,病因不明。提高对本瘤认识可避免误诊更多还原

【Abstract】 Purpose To study the clinicopathological features of epithelioid hemangioendothelioma(EHE). Methods Six cases of EHE were examined with histologic, histochemical and immunohistochemical staining, in which 1 case was with ultrastructural observation. Results The patients’s age ranged from 26 to 66 years (mean 38 years). EHE presented at a variety of sites including head and neck (4 cases), upper limbs (1 case) and perineum(1 case). The morphological appearance of the tumor was characterized by epithelioid or histiocytoid tumor cells arranged in nests,cords and lumens, primitive lumen formation within a single tumor cell, and lack of mitotic activity. There were small focal necrosis and pleomorphism and the tumor cells were embedded in myxoid or hyaline matrix in some cases. AB/PAS (pH 1 0) stain showed that tumor cells were negative, myxoid stroma was light blue in 5 cases. Reticular fiber stain showed that there were many vascular lacunae. Immunohistochemical staining found that tumor cells were positive for vimentin and CD34 (6/6), negative for FⅧRAg, CK , CEA, EMA and MAC387. Ultrastructural study demonstrated Weibel Palade bodies in the cytoplasm. Conclusions EHE is a distinctive low grade tumor whose etiology remains unknown. Well knowing of the EHE can avoid confusion with other similar lesions.更多还原