原发性肺动脉肉瘤的诊断和治疗

Diagnosis and Treatment of Primary Pulmonary Artery Sarcoma

目的提高对原发性肺动脉肉瘤(PAS)的认识和早期诊治水平。方法结合北京安贞医院2001年10月至2010年5月手术病理确诊的4例PAS患者的临床资料和国外有关文献,对原发性肺动脉肉瘤的临床特点进行总结分析。结果原发性肺动脉肉瘤患者主要表现为活动性呼吸困难、气短和胸闷,休息可缓解,和肺血栓栓塞症相似,但前者无下肢深静脉血栓形成,胸部增强CT肺动脉造影多显示肺动脉扩张,主肺动脉及左、右肺动脉内大块充盈缺损,管腔外浸润阴影,溶栓、抗凝治疗无效等,确诊需手术。手术成功治疗后症状多明显缓解,术后辅以放疗和/或化疗可提高疗效。结论原发性肺动脉肉瘤临床上罕见,临床表现和肺血栓栓塞症相似,易误诊为肺血栓栓塞症而给予溶栓抗凝治疗。胸部增强CT肺动脉造影及手术病理有助明确诊断,手术切除是主要治疗方法。

Objective To improve the early diagnosis and treatment of primary pulmonary artery sarcoma.Methods Clinical data of 4 patients with pulmonary artery sarcoma in Beijing Anzhen Hospital from October 2001 to May 2010 were retrospectively analyzed and related literature were reviewed.Results Primary pulmonary artery sarcoma mainly presented dyspnea,shortness of breath,and chest tightness at exertion.These presentations were similar to pulmonary thromboembolism but not accompanied by deep venous thrombosis.Chest contrast-enhanced CT angiography revealed pulmonary artery enlargement,massive filling defect in main pulmonary artery and left or right pulmonary artery as well as extraluminal extension shadow.Thrombolytic and anticoagulant therapy had no clinical effect.An operation was needed to confirm the diagnosis.The symptoms often relieved after successful surgery.Postoperative adjuvant treatment including radiotherapy or chemotherapy could improve prognosis.Conclusions Primary pulmonary artery sarcoma is a rare entity which has similar clinical presentations to pulmonary thromboembolism,so it is usually misdiagnosed as pulmonary thromboembolism.Chest contrast-enhanced CT angiography and operative pathologic examination are helpful for the diagnosis.Surgical resection is the main therapy.