中文摘要：

目的 比较分析肺动脉肉瘤（PAS）的临床影像学特征和外科治疗效果.方法 回顾性分析2001年11月至2014年1月安贞医院心脏外科中心收治19例PAS的诊断和治疗结果.结果 19例患者的肺动脉CT血管造影（CTA）均显示蚀壁征;14例手术后病理学检查证实为PAS;5例未行手术治疗者PET-CT检查显示氟脱氧葡萄糖摄取异常增高团块影.14例手术中,肺动脉内膜剥脱术12例,一侧全肺切除2例;5例未手术.手术患者无围手术期死亡.5例未手术患者出院后平均生存（20.3 ±ll.2）d,14例手术患者术后平均生存（16.8±3.8）个月,差异有统计学意义（P=0.000）.不同术式和不同病理分型的PAS生存时间差异均无统计学意义.9例未行辅助性放疗化疗患者生存时间为（12.3±3.2）个月,5例行辅助性放疗化疗患者生存时间为（22.8±4.3）个月,差异有统计学意义（P =0.000）.结论 “蚀壁征”具有PAS疾病特征性;外科手术治疗可延长PAS的生存期,辅助性化疗和放疗可进一步延长患者的生存期.

英文摘要：

Objective To characterize the differential diagnostic characteristics and the surgical treatment efficacy of pulmonary artery sarcoma （PAS）.Methods From November 2001 to January 2014,19 PAS patients were diagnosed and 14 of them underwent surgery at Beijing Anzhen Hospital.And their data were retrospectively reviewed.Results All 19 patients underwent pulmonary artery computed tomography angiography （CTA） scan.All showed a filling defect within the lumen of pulmonary artery with a sign of wall eclipsing.And 14 of them had pulmonary artery sarcoma confirmed through postoperative histopathological examination while another 5 patients were confirmed to have FDG abnormal high intake mass shadow on Positron emission tomography-computed tomography （PET-CT） scan.Fourteen patients underwent surgery,including pulmonary endarterectomy （n =12） and pneumonectomy （n =2）,and another five had no indication for operation and died shortly.No perioperative death occurred for surgical patients.Five non-surgical patients survived （20.3 ± 11.2） days after discharge.And 14 postoperative patients survived （16.8 ± 3.8） months.The difference between two groups reached statistical significance （P =0.000）.The survival difference between two surgical procedures and between two pathological classifications did not reach statistical significance.Nine patients did not while another 5 received adjuvant radiotherapy and chemotherapy.Their average survivals were （12.3 ± 3.2） and （22.8 ± 4.3） months respectively.And the inter-group difference reached statistical significance （P =0.000）.Conclusions The sign of wall eclipsing on pulmonary artery CTA scan is pathognomonic for PAS.Radical surgical resection provides a longer survival than non-surgery and adjuvant chemotherapy may further extend survival.