摘要目的 探讨肺恶性血管周上皮样细胞肿瘤(malignant perivascular epithelioid cell tumor,PEComa)的病因、临床表现、诊断、鉴别诊断、治疗及预后.方法 回顾性分析山东大学附属省立医院呼吸科2014年9月收治的1例肺恶性血管周上皮样细胞肿瘤患者的临床资料并进行相关文献复习.以“肺恶性血管周上皮样细胞肿瘤”为检索词检索万方数据库和中国期刊全文数据库,以“pulmonary malignant perivascular epithelioid cell tumor”和“PEComa”为检索词检索PubMed、Embase、Ovid及Cochrane数据库,检索时间为1975年1月至2015年7月.结果 患者女,50岁,因“咳嗽、胸闷憋气60 d,咯血40 d,发热7d”于2014年9月4日入院,患者主要症状为咯血和呼吸困难.CT表现为双肺弥漫分布且大小不等的结节并周围明显渗出影.行TBLB,病理诊断为肺恶性PEComa.确诊后患者拒绝进一步治疗,病情急剧进展,于确诊2周后死于呼吸衰竭.共检索到肺恶性PEComa相关文献8篇,均为个案报道.8例中男3例,女5例,年龄50～79岁,平均63岁.胸部影像学表现多为肺部边界清晰的圆形团块影.临床表现无特异性.结论 肺恶性PEComa罕见,对于快速进展的双肺弥漫结节并咯血的患者应考虑到肺恶性PEComa的可能,TBLB是一种有效的诊断方法.

abstractsObjective To analyze the pathogenesis,clinical features,diagnosis and differential diagnosis of primary perivascular epithelioid cell tumor(PEComa).Methods The clinical features,auxiliary examinations and diagnosis of a case with rapidly progressive pulmonary malignant PEComa were reported and the related literatures were reviewed.The literature review was carried out respectively in Wanfang Data,CNKI and PubMed from Jan.1975 to Jul.2015 with" pulmonary malignant perivascular epithelioid cell tumor" and" PEComa" being the search terms.Results A 50 year-old female patient was admitted to the hospital on September 4,2014 because of cough and dyspnea for 60 days,hemoptysis for 40 days and fever for 7 days.Chest CT scan showed diffuse small nodules with infiltrative border and multiple pure and mixed ground-glass opacity.Transbronchial lung biopsy (TBLB) was performed and the pathological study confirmed the diagnosis of primary pulmonary malignant PEComa.The patient declined further specific therapy,but followed by rapidly progressive respiratory failure,and died two weeks after the diagnosis.A total of 8 literatures were retrieved from Wanfang Data,CNKI and PubMed and all of them were case reports.There were 3 male and 5 female patients,aging from 50 to 79 years.Radiographically,the previously reported cases presented as round and well-circumscribed masses with or without multiple nodules in both lungs.The symptoms had no specificity.Conclusions Pulmonary malignant PEComa is a rare disease.It is easily misdiagnosed because of non-specific clinical and imaging manifestations.The final diagnosis depends on pathological biopsy.TBLB is an effective diagnostic method.