中文摘要：

目的回顾性分析原发性肺动脉肉瘤（PAS）的临床特点，提高对该病的认识。方法对2001年11月至2012年11月于北京安贞医院经手术及病理确诊的PAS患者的临床表现、实验室、影像学、病理及随访情况等进行回顾性分析，采用Kaplan-Merier估计法绘制生存曲线估测生存率。结果（1）9例患者中男性5例，女性4例，平均年龄（46±11）岁。临床表现为胸闷8例，心悸、晕厥、咳嗽、体质量下降均为2例，胸痛l例。（2）实验室检查提示9例患者的乳酸脱氢酶（LDH）、高敏C反应蛋白（hs-CRP）、红细胞沉降率（ESR）均高于正常值，D-二聚体均正常。（3）影像学检查：本组患者中7例行螺旋CT肺动脉成像（CTPA），结果显示主肺动脉干充盈缺损6例，左肺动脉主干充盈缺损6例，右肺动脉主干充盈缺损7例。本组患者中7例行超声心动图检查显示6例发现右心室扩大，有心室前后径平均（38．54±16．30）illtn；7例有心房增大，右心房横径平均（55．11±5．45）turn；7例j尖瓣关闭不全，估测肺动脉估测压力平均（81．14±21．17）mmHg（1mmHg=0．133kPa），5例诊断肺动脉血栓。4例患者行下肢深静脉超声，1例见下肢深静脉血栓形成。本组患者4例术中留置漂浮导管，并记录其术前、术后平均肺动脉压（PAP），术前PAP平均（52．00±5．23）mmHg，术后平均（23.00±5．60）mmHg。（4）9例患者均接受手术切除治疗，术中病理结果均为内膜肉瘤，其中3例平滑肌分化，1例平滑肌分化伴血管分化，1例未分化。（5）9例患者术后1、3或5个月的生存率分别是71．4％、53．6％和35．7％，中位生存时间5个月。结论对于初诊时即出现右心房、右心室扩大，有心功能不全，胸部影像学检查病变累及主肺动脉干及左右肺动脉千，且D-二聚体正常者，应高度警惕PAS。

英文摘要：

Objective To investigate the clinical characteristics of pulmonary artery sarcoma （PAS） and to improve doctors＇ awareness and the early diagnosis of this disease. Methods The clinical data of 9 cases confirmed by operation and pathology of PAS from November 2001 to November 2012 in Beijing Anzhen Hospital were reviewed. The clinical characteristics, laboratory tests, imaging manifestation, pathology as well as follow-up were studied. Survival was determined by the Kaplan-Merier method. Results （ 1 ） Main clinical manifestations were chest distress （ 8/9 ） , palpitation （ 2/9 ）, syncope （ 2/9 ）, cough （2/9） , weight loss （ 2/9 ） and chest pain （ 1/9 ） . （ 2 ） Lactate dehydrogenase （ LDH ） , high-sensitivity C-reactive protein（hs-CRP） ,erythrocyte sedimentation rate（ESR） were all elevated to different extents, but D-Direct remained in the normal range. （ 3 ） Seven cases received CTPA examination and evidenced filling defect in the main pulmonary artery trunk （6/7）, left pulmonary artery （6/7）, right pulmonary artery （7/7）. 7 cases received TrE examination and showed enlarged right ventricle（6/7） ： mean right ventricular end-diastolic diameter was （38.54 ± 16. 30）mm; enlarged right atrium （7/7） ： mean right atrium diameter was （ 55.11 ± 5.45 ） mm ; and tricuspid insufficiency （ 7/7 ） ： estimated mean pulmonary artery pressure was（81.14-＋ 21.17） mmHg（ 1 mmHg = 0. 133 kPa ）, and pulmonary stenosis （5/7）. Four cases received deep venous ultrasound examination and deep venous thrombosis （DVT） was found in 1 patient. Four cases received Ganz catheter examination and the preoperative and postoperative mean pulmonary artery pressure was （52. 00 ± 5.23 ） mmHg and （23.00 ± 5.60） mmHg, respectively . （4） All 9 patients received surgery and intimal sarcoma was diagnosed in all of them. Leiomyosarcomas was diagnosed in 3 cases, leiomyo- angiosarcoma was diagnosed in 1 case, undifferentiated sarcomas was