肺动脉肉瘤4例临床分析

Clinical analysis of 4 cases of pulmonary artery sarcoma

目的 分析4例肺动脉肉瘤(PAS)患者的临床特征,探讨手术治疗效果及预后。方法 回顾性分析2018年1月—2019年12月阜外华中心血管病医院诊治的4例PAS患者临床资料。结果 4例PAS患者,男2例、女2例,年龄35~67岁,均为慢性起病伴有急性加重;入院时均有不同程度胸闷、胸痛、呼吸困难、咳嗽、咳血等。例1和例3患者血清D-二聚体水平正常(0.30、0.25 mg/L),例2和例4患者血清D-二聚体水平升高(1.36、0.63 mg/L)。4例双下肢深静脉超声均未见异常,超声心动图检查均提示肺动脉内团块状回声改变伴肺动脉高压,肺动脉CT血管成像均提示主肺动脉及其分支血管腔内充盈缺损。4例患者院外均误诊为肺动脉血栓栓塞症,均有抗凝治疗史。例3患者行肺动脉肿物穿刺活检组织病理检查确诊为PAS后放弃治疗,出院后3个月死亡。余3例行肺动脉内膜剥脱术治疗,例2同期行肺动脉瓣置换术,例4同期行肺动脉瓣置换术及右心室流出道肿瘤切除术,术后组织病理均提示为PAS,均未再行放化疗及免疫治疗。随访至2022年10月,例1患者术后18个月因肿瘤复发致呼吸衰竭死亡,例2和例4患者肿瘤未复发转移。结论 PAS患者较少发生下肢静脉血栓和血清D-二聚体水平升高,其他临床特征不易与肺动脉血栓栓塞症鉴别,行肺动脉内膜剥脱术治疗可恢复肺动脉功能,延长生存期。

Objective To analyze the clinical characteristics of pulmonary artery sarcoma(PAS),and to investigate the therapeutic effect and prognosis.Methods The clinical data of 4 patients with PAS diagnosed and treated in Fuwai Central China Cadiovascular Hospital from January 2018 to December 2019 were retrospectively analyzed.Results In 4 patients with PAS,there were 2 males and 2 females,aged from 35 to 67 years.All of them had chronic onset followed by acute exacerbation.All patients were found chest tightness,chest pain,dyspnea,cough,hemoptysis and so on in different degrees on admission.The D-dimer level was normal in patient 1(0.30 mg/L) and patient 3(0.25 mg/L),and increased in patient 2(1.36 mg/L) and patient 4(0.63 mg/L).No abnormal ultrasonography was found in deep veins of both lower extremities.Echocardiography showed mass echo changes in pulmonary artery.Pulmonary CT angiography showed filling defects in the main pulmonary artery and its branches.All 4 patients were misdiagnosed with pulmonary thromboembolism and received anticoagulation therapy before admission.Patient 3,who was diagnosed with PAS by pulmonary artery mass biopsy,abandoned treatment and died 3 months after discharge.The other 3 patients were performed pulmonary endarterectomy,among whom patient 2 received pulmonary valve replacement,and patient 4 received pulmonary valve replacement plus right outflow tract tumor resection at the same time.The histopathological results confirmed PAS in these 3 patients,and none of them received radiotherapy,chemotherapy or immunotherapy.Till October 2022,patient 1 died of respiratory failure due to tumor recurrence 18 months after operation,and patient 2 and 4 had no tumor recurrence or metastasis.Conclusions Lower extremity venous thrombosis is less common in PAS patients,and the serum D-dimer level is normal.The other clinical features are not easy to be distinguished from pulmonary thromboembolism.Pulmonary endarterectomy can restore pulmonary artery function,and prolong the survival of patients with PAS.