肺动脉肉瘤8例临床分析  

Clinical Analysis of Eight Patients with Pulmonary Artery Sarcoma

作　　者：梁颖[1] 米玉红[1] 高元明[2] 刘双[2] 黄小勇[3] 杨赓[1] 方微[4] 甘辉立[5] 

机构地区：[1]首都医科大学附属北京安贞医院心肺血管抢救中心EICU,北京100029 [2]首都医科大学附属北京安贞医院呼吸科,北京100029 [3]首都医科大学附属北京安贞医院影像科,北京100029 [4]首都医科大学附属北京安贞医院病理科,北京100029 [5]首都医科大学附属北京安贞医院心外科,北京100029

出　　处：《中国呼吸与危重监护杂志》2013年第3期284-288,共5页Chinese Journal of Respiratory and Critical Care Medicine

摘　　要：目的提高对肺动脉肉瘤(PAS)的认识和早期诊断。方法总结分析北京安贞医院2001年4月至2012年4月明确诊断的8例PAS临床资料。结果 8例PAS患者中男5例,女3例,男女比例为1.67∶1。年龄32～67岁,平均(46.75±11.47)岁。临床表现主要有活动性胸闷气短、间断性晕厥、活动后心悸等。实验室检查提示PAS患者多无明显低氧血症和D-二聚体升高。超声心动图(UCG)提示肺动脉内团块状回声、肺动脉高压、右心扩大等。双下肢深静脉超声正常。胸片提示肺动脉段突出、右肺动脉段饱满、右肺门增大、胸腔积液等。CT肺动脉造影(CTPA)提示肺动脉扩张,多累及主肺动脉和左、右肺动脉,多合并心包积液、胸腔积液;肺通气/灌注显像提示双肺血流灌注及通气显像不匹配,可见多个肺段及亚肺段放射性稀疏和缺损,累及3～13个肺段。肺动脉造影表现多为主肺动脉和左、右肺动脉充盈缺损。病理学检查结果:8例患者均经术后病理证实,3例支持平滑肌肉瘤分化,2例倾向于未分化肉瘤,3例未进行病理分型。8例患者术前均被误诊为肺血栓栓塞症(PTE),误诊时间平均(85.6±21.5)d。8例患者中7例单纯手术切除治疗,1例行手术切除加放、化疗。病情缓解出院7例,死亡1例。结论 PAS临床罕见,极易误诊,临床医师应提高对此病的认识,以早期诊断和治疗,延长生存期。Objective To improve the knowledge of pulmonary artery sarcoma （PAS） and early diagnosis. Methods The clinical data of 8 patients with PAS confirmed by biopsy from April 2001 to April 2012 in Beijing Anzhen Hospital were retrospectively analyzed. Results There were 5 males and 3 females, with mean age of 46.75 ±11.47 years [ range：32-67 years ]. The main clinical manifestations were chest tightness, shortness of breath, intermittent syncope, heart palpitations at exertion, etc. Laboratory examinations showed the patients with PAS have no obvious hypoxemia and most of them have normal D-dimer level. Echocardiography revealed pulmonary hypertension,right ventricular enlargement, and echo of massive lumps in main pulmonary truck. Lower limb veins were normal in color doppler uhrasonography. Chest X-ray revealed prominent pulmonary artery segment, full segment of the right pulmonary artery, an increased hilum and pleural effusion. CT pulmonary angiography showed expansion of pulmonary artery,large filling defect in main pulmonary truck and left or right pulmonary artery, combined with pericardial effusion, pleural effusion. Lung ventilation/perfusion imaging did not match, showing radioactive sparse and defects in multiple lung segments and subsegments,involved 3 to 13 lung segments. Pulmonary angiography showed filling defects in the main pulmonary artery, left or right pulmonary artery. 8 patients were confirmed pathologically after operation. Pathological results showed leiomyosareoma differentiation in 3 cases, undifferentiated sarcoma in 2 cases, and undefined pathological type in 3 cases. All 8 patients were misdiagnosed as pulmonary embolismbefore surgery. The average days of misdiagnosis were 85.6 ± 21.5 days. 7 cases were given simple surgical resection, one case underwent surgical resection combined with radiotherapy and chemotherapy. 7 cases were relieved and discharged, and one case died. Conclusion PAS is a rare disease clinically and is easily misdiagnosed as pulmonary embolism. Clinicians shoul

关 键 词：肺动脉肉瘤 诊断 治疗 

分 类 号：R734.2[医药卫生—肿瘤]