摘要目的 探讨软组织血管肉瘤肺转移与原发性肺血管肉瘤的鉴别要点.方法 通过对1例小腿血管肉瘤肺转移患者的误诊、纠正诊断、治疗过程的分析,结合文献复习总结原发性及转移性肺血管肉瘤特点.以"pulmonary angiosarcoma"和"lung angiosarcoma"为检索词在PubMed数据库进行检索,以"肺血管肉瘤"为检索词在中国知网数据库及万方数据库中检索,检索时间为1950—2015年.结果 患者男,39岁,因"咳嗽、咳痰、咯血10个月"于2013年9月到本院就诊,最初经肺楔形切除活检诊断为"原发性肺血管肉瘤",但患者在22个月后出现小腿疼痛不适,最终通过全身正电子发射计算机断层显像扫描及小腿穿刺活检确诊为小腿原发性软组织血管肉瘤伴多发肺转移.检索文献,纳入23篇资料完整(包括病史、诊疗过程及随访结果)的原发肺血管肉瘤文献,共26例患者,男18例,女8例,年龄19~85岁,平均(52±18)岁.原发性肺血管肉瘤主要表现为单发或多发肺内实性结节或肿块,伴或不伴磨玻璃影.本例患者胸部CT示多发的薄壁空洞和磨玻璃影以及反复发生的气胸,而文献中原发性肺血管肉瘤肺部影像学则没有这些表现.结论 软组织血管肉瘤肺转移的胸部影像不同于原发性肺血管肉瘤,前者多表现为薄壁空洞和气胸,联合正电子发射计算机断层显像全身扫描有助于鉴别原发性血管肉瘤及血管肉瘤肺转移.

abstractsObjective To investigate the differential diagnosis between pulmonary metastases from soft-tissue angiosarcoma and primary pulmonary angiosarcoma .Methods A case of soft-tissue angiosarcoma with pulmonary metastases was reported and related literatures were reviewed .Results A 39 year-old man complaining of hemoptysis, cough, and sputum for 10 months was admitted to our hospital in September 2013.He was initially diagnosed as having primary pulmonary angiosarcoma after wedge -resection biopsy of the lung.After 22 months since onset , he felt discomfort in his leg , which led to the confirmative diagnosis of soft-tissue angiosarcoma of the leg with multiple pulmonary metastases by a full-body PET/CT scan and core needle biopsy of the leg . Twenty-three articles concerning primary pulmonary angiosarcoma with complete records of history ,treatment and follow-up of patients were included in the literature review .A total of 26 patients were reported in these articles , including 18 males, 8 females, age 19-85 years, average (52 ±18) years.Primary pulmonary angiosarcoma was mainly manifested as single or multiple pulmonary nodules or masses , with or without ground glass opacity .In our case , chest CT showed multiple thin-wall cysts and ground glass opacities , and recurrent spontaneous pneumothorax , which had never been reported in literatures on primary pulmonary angiosarcoma . Conclusions Pulmonary metastases from soft-tissue angiosarcoma differed from primary pulmonary lesions in terms of chest imaging , with the former usually showing thin-wall cysts and pneumothorax . A full-body PET-CT was essential for differential diagnosis between primary and metastatic pulmonary angiosarcoma .