原发性肺及胸壁上皮样血管内皮瘤3例并文献复习

Three cases report and literature review of lung and chest wall primary epithelioid hemangioendothelioma

目的总结肺及胸壁上皮样血管内皮瘤的诊断与治疗经验。方法回顾性分析3例肺及胸壁上皮样血管内皮瘤患者的临床资料并复习文献。结果 3例肺及胸壁上皮样血管内皮瘤临床表现缺乏特异性,3例均行手术治疗。石蜡病理示上皮样血管内皮瘤,免疫组织化学检查CD34(+),SMA(+),S-100(-)。2例术后1个月复查胸片未见异常,1例术后失访。结论肺及胸壁上皮样血管内皮瘤缺乏特异性的检查方法,以及有效的治疗手段,主要采用以外科和化疗为主的综合治疗。

Objective To explore the experience of diagnosis and treatment of lung and chest wall primary epithelioid hemangioendothelioma.Methods Three cases were analyzed and the related literatures were reviewed.The clinical manifestations,diagnosis,pathological and biological characteristics,treatment of lung or chest wall primary epithelioid hemangioendothelioma were described.Results It was almost impossible to make a definite diagnosis before operation for the lack of specific clinical manifestations.The final diagnosis relied on pathological analysis and immunohistochemistry stains.There was no effective treatment for it and the prognosis was unpredictable.Conclusions As a kind of rare diseases,lung or chest wall primary epithelioid hemangioendothelioma is hard to diagnose and treat.Target therapy or transplantation may be the future approach.