肺上皮样血管内皮细胞瘤临床病理分析

目的分析肺上皮样血管内皮瘤(PEH)的临床病理特征、诊断、鉴别诊断、治疗及预后。方法对1例原发性PEH的临床病理和免疫组织化学特征进行回顾性分析,并结合相关文献复习。结果 CT检查示双肺多发性结节,行肺部肿物切除术。光镜下肿瘤为境界清楚的嗜酸性结节,中心可见均一的类似淀粉样变或软骨瘤的透明变性组织;瘤细胞呈上皮样,也可呈实性巢状,瘤细胞出现脂肪样空泡,有的空泡内含单个红细胞,实为新生的原始血管;细胞异型不明显,核分裂像罕见。免疫组化结果:肿瘤组织中表达CD34、CD31、Ⅷ因子、Vimentin、CK等标记物。结论 PEH是1种罕见的低度恶性肿瘤,好发于中年女性,临床表现无明显特异性,诊断该病需结合影像学及病理学相关检查。

Clinicopathological Analysis of Pulmonary Epithelioid Hemangioendothelioma

Objective To analysis the clinicopathological features、diagnosis and differential diagnosis、treatment and prognosis of Pulmonary epithelioid hemangioendothelioma（PEH）.Methods The clinicopathological and immunohistochemical data of one patient with PEH were retrospectively analyzed,and the related literatures were reviewed.Results Chest CT scan showed multiple nudules in both lobes of the lungs.The patient was treated by resection of the lung lobes.Microscopically,the tumor was a well-defined and eosinophilic nodules,homogeneous amyloid or hyaline degeneration of chondroma could be found in central area of nodules;The tumor cells were epithelioid or solid nests-like.Tumor cell can appear fat-like emptily-bubble,some of which was a real original and newborn blood vessel containing single blood cell.Cell atypia was not obvious and mitotic figures were rare.Immunohistochemically,the tumor cells were positive for CD34,CD31,Ⅷ Factor and other markers.Conclusion PEH is a rare kind of low malignant tumor that easily occur in women.PEH has no specific clinical features,and diagnosis should be made in combination with image and histological examination.