Gan Huili, Zhang Jianqun, Feng Lei, Zhang Zhitai, Liang Lin, Zhu Guangfa, Chen Dong
Department of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart, Lung & Vascular Disease, Beijing 100029, China. Email:
Zhonghua Yi Xue Za Zhi. 2014 Apr 29;94(16):1252-4.
To characterize the differential diagnostic characteristics and the surgical treatment efficacy of pulmonary artery sarcoma (PAS).
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.
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).
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.
探讨肺动脉肉瘤（PAS）的鉴别诊断特点及外科治疗效果。
回顾性分析2001年11月至2014年1月在北京安贞医院确诊的19例PAS患者资料，其中14例行手术治疗。
19例患者均行肺动脉CT血管造影（CTA）检查，均显示肺动脉腔内充盈缺损及管壁受压征象。14例经术后病理检查确诊为肺动脉肉瘤，5例经正电子发射断层扫描-计算机断层扫描（PET-CT）检查显示氟代脱氧葡萄糖（FDG）摄取异常增高的肿块影。14例患者行手术治疗，其中肺动脉内膜剥脱术12例，肺切除术2例；另外5例无手术指征，短期内死亡。手术患者无围手术期死亡。5例非手术患者出院后存活（20.3±11.2）天，14例术后患者存活（16.8±3.8）个月，两组差异有统计学意义（P = 0.000）。两种手术方式及两种病理类型之间的生存差异无统计学意义。9例患者未接受辅助放化疗，5例接受辅助放化疗，其平均生存期分别为（12.3±3.2）个月和（22.8±4.3）个月，组间差异有统计学意义（P = 0.000）。
肺动脉CTA扫描显示的管壁受压征象是PAS的特征性表现。根治性手术切除较非手术治疗能延长生存期，辅助化疗可进一步延长生存期。
