肺上皮样血管内皮瘤的临床病理观察

Clincopathologic study on pulmonary epithelioid hemangioendothelioma

目的探讨肺上皮样血管内皮瘤(pulmonaryepithelioidhemangioendothelioma,PEH)的临床病理特征、诊断及鉴别诊断。方法对3例PEH进行光镜观察和免疫组化标记,并结合文献进行分析。结果镜下见肿瘤细胞具有上皮样或组织细胞样形态,瘤细胞为圆形或多角形,呈小巢状、索状甚至腺样结构或不规则状,分布于黏液间质中;间质可见少量黏液样变或玻璃样变;瘤细胞内含有原始血管腔,核分裂象、多形性及坏死少见。肿瘤细胞表达CD34、FⅧRAg、CD31等血管内皮细胞标记,部分病例同时表达CK和(或)Vim。结论PEH是一种低度恶性肿瘤,其病理形态具有一定的特征性,诊断时需要与肺转移癌、肺上皮样血管肉瘤、肺淋巴管肌瘤病鉴别。

Purpose To study the clincopathologic morphological character and diagnosis and differential diagnosis of pulmonary epithelioid hemangioendothelioma（PEH）. Methods Three cases of PEH were investigated with routine histologic and immunohistochemical staining, together with review of the literature. Results Tumor cells were ＂epithelioid＂ or ＂histiocytoid＂ ;rounded or polygonal tumor cells arranged in nests, cords, glandulose feature and irregular patterns;the tumor cells were embedded in myxoid or hyaline matrix. Primitive lumen formation within a single tumor cell （intra cytoplasmic lumens） ;The kind of tumor lacks of mitoticactivity, pleomorphism, and necrosis; Immunohistochemical analysis showed that the tumor cells expressed sensitive markers of vascular endothelial cells, such as CD34, factor Ⅷ and CD31. Expression of cytokeratin and vimentin were also detected in some cases. Conclusions PEH is a distinctive low grade sarcoma which has distinctive histopathologic features, but it should be differentiated from metastatic cancer within lung, pulmonary eptheloid hemanglosarcoma and pulmonary lymphangloleiomyomatosis.