肺上皮样血管内皮瘤1例并文献复习  

Clinical analysis of pulmonary epithelioid haemangioendothelioma: one case report and literature review

作　　者：张雯[1] 罗莉[1] 唐雪峰[2] 吴国明[1] 王斌[1] 徐瑜[1] 

机构地区：[1]第三军医大学新桥医院全军呼吸内科研究所,全军呼吸病研究重点实验室,重庆400037 [2]第三军医大学新桥医院病理科,重庆400037

出　　处：《中华肺部疾病杂志（电子版）》2015年第2期37-40,共4页Chinese Journal of Lung Diseases(Electronic Edition)

基　　金：国家自然科学基金(30900657;31371013)

摘　　要：目的探讨肺上皮样血管内皮瘤（PEH）的临床特征,提高对该病的诊疗水平。方法通过1例无远处转移的PEH的病例报道,并结合国外文献回顾,分别就PEH的临床表现、影像学、组织病理及免疫组化表型等进行总结分析。结果包括本例患者在内共国内外报道了45例PEH,其中女性33例（73.3%）,男性12例（26.7%）,发病年龄10~76岁;无自觉症状26例（39.4%）,呼吸困难16例（24.2%）,咳嗽10例（15.2%）,胸痛8例（12.2%）;影像学表现为双肺多发结节37例（82.2%）,单肺多发结节5例（11.1%）,孤立性结节3例（6.7%）;文献中远处转移的病例36例（92.3%）,无远处转移3例（7.7%）,最常见的转移部位有骨骼、肝脏、脾脏等。45例患者的FactorⅧ、CD31、CD34、Fli-1的阳性表达率分别为88.9%、97.1%、97.3%、100%。结论 PEH是一种罕见的血管源性恶性肿瘤,其临床及影像学表现缺乏特异性,确诊有赖于组织病理检测及免疫组化学。Objective To explore the clinical characteristics of pulmonary epithelioid haemangioendothelioma（ PEH） and to improve diagnosis and treatment level of PEH. Methods It was analyzed that the clinical manifestation,computed tomography scan,immunohistochemistry of PEH through a case reported and English literatures reviewed. Results There were 45 cases admitted in our study totally,including 33 women（ 73. 3%） and 12 men（ 26. 7%）. The age of onset was 10-76 years. Although nearly 40%of the patients were asymptomatic（ n = 26, 39. 4%） including our patient, many other non-specific presentations were described such as dyspnoea（ n = 16,24. 2%）,cough（ n = 10,15. 2%）,chest pain（ n = 8,12. 2%）. Radiologically,PEH usually presented as nodular shadows. Most of the nodules were multiple（ 93. 3%）. They were distributed bilaterally（ n = 37,82. 2%）,unilaterally（ n = 5,11. 1%） or less frequently solitary（ n = 3,6. 7%）. Distant metastases were confirmed in 36 patients（ 92. 3%）,no metastasis in 3 patients（ 7. 7%）, the common sites involved including the bone, liver, spleen, etc.Immunohistochemistry revealed that all tumors displayed immunoreactivity to some or all of the vascularendothelial markers, including Factor Ⅷ（ 88. 9%）, CD31（ 97. 1%）, CD34（ 97. 3%）, Fli-1（ 100%）. Conclusions PEH is a rare malignant vascular tumor. It’ s clinical manifestation and radiologic presentation are non-specific. The positive diagnosis is based on the analysis of histopathology and Immunohistochemistry.

关 键 词：肺上皮样血管内皮瘤 临床病理特征 治疗 预后 文献复习 

分 类 号：R734.2[医药卫生—肿瘤]