原发性肺血管肉瘤1例并文献复习

Primary pulmonary angiosarcoma:a case report and literature review

目的分析原发性肺血管肉瘤的临床症状、影像学表现、病理特征、治疗方案及预后等要点,以提高临床诊疗能力。方法通过对1例原发性肺血管肉瘤患者临床诊断及治疗过程的分析,并结合相关文献总结复习原发性肺血管肉瘤的特点。以"lung angiosarcoma""pulmonary angiosarcoma""肺血管肉瘤"为关键词分别在PubMed、中国万方、中国知网数据库中检索,时间为1992年至2020年。结果患者,女,47岁,因"右侧胸痛10余天"于2020年3月28日入住山东大学齐鲁医院呼吸与危重症医学科,最终经CT引导下肺穿刺活检确诊为肺血管肉瘤。经过筛选,共纳入30篇资料完整(包括病史、诊断、治疗、预后)且患者最后确诊为原发性肺血管肉瘤的文献,共36例患者,其中男25例,女11例,18~81岁,平均(52±17)岁。原发性肺血管肉瘤临床症状主要包括咳嗽、咯血、胸痛、呼吸困难等,影像学常见为单发或多发结节或肿块,伴或不伴磨玻璃样改变、胸腔积液、血管充盈缺损等。最终诊断依赖于组织病理及免疫组织化学检查。结论原发性肺血管肉瘤罕见,临床工作中应对咯血、胸痛、肺部结节/肿块等表现提高警惕,及早取得病理学诊断,避免误诊,正确治疗,延长生存期。

Objective The clinical symptoms,imaging manifestations,pathological features,treatment and prognosis of primary pulmonary angiosarcoma were summarized in order to enhance the clinical diagnosis and treatment.Methods The clinical diagnosis and treatment of one case of pulmonary angiosarcoma were analyzed,and the characteristics of primary pulmonary angiosarcoma were reviewed.Key words including"lung angiosarcoma"and"pulmonary angiosarcoma"were searched in PubMed,China Wanfang and CNKI databases from 1992 to 2020.Results A 47 year-old female patient complaining of right chest pain for more than ten days was admitted to our department on March 28,2020.Pulmonary angiosarcoma was diagnosed with CT-guided percutaneous lung biopsy.Altogether 30 studies with complete data(including history,diagnosis,treatment and prognosis)were retrieved,involving 36 patients,including 25 males and 11 females,aged 18-81 years,average(52±17)years.The clinical symptoms of primary pulmonary angiosarcoma included cough,hemoptysis,chest pain and dyspnea.The imaging characteristics were single or multiple nodules or masses with or without ground-glass opacity,pleural effusion and filling defects.The diagnosis depended on histopathology and immunohistochemistry.Conclusion Primary pulmonary angiosarcoma is a rare disease,with manifestations such as hemoptysis,chest pain,and pulmonary nodules/masses.Early diagnosis and appropriate treatment can prolong patients'survival.