原发性肺动脉肉瘤误诊误治原因分析

目的 提高对原发性肺动脉肉瘤的认识,减少误诊、误治.方法 结合北京安贞医院2001年10月至2009年3月手术病理证实的4例原发性肺动脉肉瘤患者的的临床资料和国外有关文献,对原发性肺动脉肉瘤的误诊、误治原因进行分析.结果 原发性肺动脉肉瘤的临床表现和肺血栓栓塞症相似,主要有活动性呼吸困难/气促、胸闷、胸痛、咳嗽、咯血、晕厥等,无特异性;彩色多普勒超声心动图显示右心扩大,三尖瓣返流.肺动脉高压,右室流出道及主肺动脉内团块样异常回声;多普勒血管超声显示下肢静脉正常;CT肺动脉造影显示肺动脉扩张,管腔狭窄,主肺动脉及左、右肺动脉内大块充盈缺损等.4例患者术前全部误诊为肺栓塞,给予溶栓抗凝治疗,疗效不佳.手术后死亡1例,3例存活者病情缓解,无一例辅助放疗和(或)化疗.结论 原发性肺动脉肉瘤临床上少见,易误诊为肺栓塞给予溶栓抗凝治疗,确诊需手术,手术为主的综合治疗是治疗此病的有效手段.

Cause analysis of mistreatment and misdiagnosis of primary pulmonary artery sarcoma

Objective To improve the understanding of primary pulmonary artery sarcoma (PAS) and reduce the rate of misdiagnosis and mistreatment. Methods The clinical data of four PAS patients confirmed with biopsy and relevant foreign literatures were analysed to explore the causes of mistreatment and misdiagnosis of PAS. Results The clinical manifestations of PAS were similar to those of pulmonary thromboembolism,mainly including exertional dyspnea/short breath, chest distress, chest pain, cough, hemoptysis,syncope, etc. , without specificity. Color Doppler echocardiography showed right ventricular enlargement, tricuspid regurgitation, pulmonary arterial hypertension and clump like abnormal echo in right ventricular outflow tract and main pulmonary artery. Doppler vascular ultrasound showed lower extremity venous was normal. CT pulmonary angiography showed expansion of pulmonary artery, luminal stenosis,and large filling defect in main pulmonary artery and left/right pulmonary artery. Four cases were all misdiagnosed as pulmonary embolism before surgery,received thrombolytic and anticoagulant therapy, but the effect was poor. One case died and three cases relieved after operation, no one case received adjuvant chemotherapy and (or) radiotherapy. Conclusions PAS is rare in clinic, and is often misdiagnosed as pulmonary thromboembolism for thrombolytic anticoagulant therapy. The confirmed diagnosis needs biopsy. Comprehensive treatment based on surgery is effective for the disease.