原发性肺动脉肉瘤11例临床分析

Clinical analysis of 11 patients with 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。方法手术病理证实的原发性肺动脉肉瘤11例,对其临床资料进行回顾性分析。结果(1)11例患者中,男4例,女7例,平均年龄44.3±9.1岁。(2)临床主要表现有活动性呼吸困难、胸痛、咳嗽、咯血、晕厥等。(3)超声心动图显示右心室扩大、肺动脉高压、右室流出道及主肺动脉内占位性病变,下肢静脉超声正常。(4)CT肺动脉造影(CTPA)表现为肺动脉扩张、主肺动脉及左、右肺动脉内肿块样阴影等。(5)11例患者术前全部被误诊,多数被误诊为肺血栓栓塞症(PTE)。(6)11例患者中10例行手术切除治疗,9例缓解出院,1例死亡;另1例内科治疗死亡。结论PAS临床上少见,易误诊为PTE。临床医师应提高认识,以早期诊断和综合治疗此病。

Objective To increase the understanding of primary pulmonary artery sarcoma（PAS） and the level of its early diagnosis and treatment. Methods The clinical data of 11 PAS patients confirmed with biopsy were retrospectively analyzed. Results （ 1 ） There were 4 males and 7 females, mean age was 44.3 ±9. 1 years. （2）Main clinical manifestations were exertional dyspnea,chest pain,cough, hemoptysis, syncope, etc. （ 3 ） Enlarged right vehicle, pulmonary arterial hypertension, massive lumps in flow tract of right vehicle and main pulmonary truck were observed in echocardiography. Lower limb veins were normal in color doppler ultrasonography. （4） CT pulmonary angiography（CTPA） showed expansion of pulmonary artery, large filling defect in main pulmonary truck and right or left pulmonary artery. （5） 11 cases were all misdiagnosed, most of them had pulmonary thrumboembofism（PTE）. （6）In the 10 cases receiving surgery,9 cases were relieved and discharged, and one died. Conclusion PAS is a rare disease clinically, and it is easily misdiagnosed as PTE. Clinicians should enhance the recognition to its early diagnose and comprehensively treat the disease.