肺恶性血管周上皮样细胞瘤一例并文献复习  

Primary pulmonary perivascular epithelioid cell tumor：a case report and literature review

作　　者：王志远[1] 李辉[1] 陈其瑞[1] 

机构地区：[1]首都医科大学附属北京朝阳医院胸外科,100020

出　　处：《肿瘤研究与临床》2010年第12期801-803,共3页Cancer Research and Clinic

摘　　要：目的分析原发性肺血管周上皮样细胞瘤（PEComa）的临床病理特点及诊断治疗。方法报道2010年4月诊治的1例肺恶性PEComa患者的症状、影像、病理、治疗及随访情况，结合文献进行分析。结果患者术前无特异临床症状，为检查发现。PEComa术前容易通过影像学手段发现但难以确诊，最终诊断依靠病理学表现和免疫组织化学。原发性肺恶性PEComa文献鲜见报道。结论肺PEComa是一种非常罕见的问叶组织肿瘤，恶性PEComa非常罕见，依靠病理学方法诊断。其生物学行为不明确，建议术后长期随访。Objective To investigate the clinicopathological features,the diagnosis and therapy of primary puhnonary perivascular epithelioid cell tumor （PEComa）. Methods One case of primary puhnonary perivascular epithelioid cell tumor diagnosed and treated in April 2010 was presented. The clinieal symptoms, imaging, pathology features, treatment and follow-up record of the patient were analyzed retrospectively,and relevant literatures were reviewed. Results The patient had not special clinical symptoms and was found through the medical checkup. The imaging methods could easily find the tumor but hard to diagnose it. The final diagnosis depended on the pathologic features and immunohistoehemistry techniques. Among present literatures, scarce cases of pulmonary malignant PEComa were reported. Conclusion Primary pulmonary PEComa is a very rare tumor that arises from mesenchymal tissues, especially the malignant cases. The diagnosis of PEComa mainly depends on the pathological features. Because of the uncertain biologic behavior pulmonary PEComa should be followed up closely for a long term.

关 键 词：肺 血管周上皮样细胞瘤 免疫组织化学 病理学 

分 类 号：R734[医药卫生—肿瘤]