上皮样血管内皮瘤临床病理学分析

目的：探讨上皮样血管内皮瘤（EH）的病理形态及免疫组化特点。方法：对5例不同部位的EH进行光镜观察和免疫组化标记，并结合文献进行讨论。结果：5例中男性2例，女性3例，年龄37－75岁，中位年龄54岁，肿瘤发生部位分别是腹壁，肺，阴茎，鼻前庭，骨，光镜下见肿瘤细胞形态多样，排列成巢状，条索状甚至腺样结构。瘤细胞短梭形，胞浆内空泡，此特征为单细胞原始管腔结构，肿瘤细胞表达血管内皮细胞标记如CD34，Ⅷ因子，UEA等，部分病例同时表达CK和／或Vim。结论：EH的病理形态具有一定的特征性，诊断时需要和转移性癌，上皮样血管肉瘤鉴别，同时在概念上需要与上皮样血管瘤区别，本病生物学行为与组织学形态有不一致性，治疗按低度恶性处理。

Epithelioid hemangioendothelioma: a clinicopathological analysis of 5 cases

Objective To study the morphological and immunohistochemical features of epithelioid hemangioendothelioma (EH). Methods Five cases of EH were investigated with routine histologic and immunohistochemical staining, together with review of the literature. The clinicopathologic characteristics of the tumor and its differential diagnosis were discussed.Results Age of the patients ranged from 37 to 75 years (mean 54), with 2 male and 3 female. The tumors located in abdominal wall, lung, penis, nasal vestibule and bone. The tumor cells were short and spindle-shaped, with intracytoplasmic vacuoles, a feature indicating primitive lumen formation by single cell, mild degree of nuclear pleomorphism and rare mitotic figures. Immunohistochemical analysis showed that the tumor cells expressed CD34, Factor VIII and UEA that were the sensitive markers for vascular endothelial cells. Expression of cytokeratin and vimentin were also detected in some cases. Conclusions EH has distinctive histopathologic features, but should be differentiated from metastatic sarcoma, epitheloid hemangioendothelial sarcoma and epithelioid hemangioma. The biologic behavior of EH is not completely consistent with its histopathologic features, therefore it should be treated as a low-grade malignancy.