摘要目的：探讨上皮样血管内皮瘤（ EHE）的临床病理学特点。方法搜集并分析13例EHE的临床病理学特征及免疫组织化学特点。结果患者男性6例，女性7例；年龄11～74岁，平均年龄43岁。肿瘤分别位于软组织、肝脏、肺、纵隔、骶骨及胸膜。镜下观察瘤细胞上皮样、梭形或多角形，呈条索状、小巢状生长，部分瘤细胞胞质空泡化，内含红细胞；瘤细胞胞质丰富，嗜酸性，间质显示不同程度黏液样变或玻璃样变。3例患者部分肿瘤细胞异型性显著，可见核分裂象及坏死形成。免疫组织化学染色显示肿瘤细胞不同程度阳性表达CD31、 FLI-1、ERG、CD34、第八因子相关抗原、广谱细胞角蛋白。10例患者获得随访，时间8～65个月，7例健在、3例死亡。结论 EHE是一种罕见的恶性血管肿瘤，除发生于软组织外，还可见于多种实质器官，镜下瘤细胞呈显著的上皮样，而易误诊为癌；熟悉其临床病理学特征合并应用血管内皮标志物免疫组织化学检查可避免误诊。

abstractsObjective To investigate the clinicopathologic characteristics of epithelioid hemangioendothelioma ( EHE) .Methods Thirteen cases of EHE were analyzed by gross examination, light microscopy and immunohistochemical staining.Results The patients included 6 males and 7 females.The age of patients ranged from 11 to 74 years ( mean=43 years) .The tumors were located in soft tissue, liver, lung, mediastinum, sacrum, and pleura.The tumor cells were epithelioid,spindled or polygonal-shaped and exhibited cord-like or small nest growth pattern.Some tumor cells formed cytoplasmic vacuoles, which contained erythrocytes.The tumor cells showed abundant eosinophilic cytoplasm.The stroma varied from highly myxoid to hyaline.In 3 cases, the tumors contained areas with significant atypia, brisk mitotic activity and necrosis.Immunohistochemical study showed that the tumor cells were positive for CD31, ERG, FLI-1, CD34, factor Ⅷ and CKpan to different extents.Follow-up information was available in 10 patients with duration ranging from 8 to 65 months.Seven patients were alive and three died.Conclusions EHE is easily a rare maligant vascular tumor,occurs in many organs other than soft tissue.EHE is misdiagnosed as carcinoma for its significant epithelioid morphology.Recognizing its clinicopathologic characteristics and combined application of specific vascular endothelial immunohistochemical markers are important to avoid confusion with other lesions.