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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 PAS patients confirmed with biopsy were retrospectively analyzed.Using the Kaplan-Merier estimate survival curves estimated survival.Results ①There were 5 males and 4 females, mean age was 46±11 years.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), etc.②lactate dehydrogenase (LDH), high-sensitivity C-reactive protein (hs-CRP), erythorcyte sedimentation rate (ESR) were all elevated to different extents, but D-Dimer was in the normal range.③7 cases received CTPA, showed filling defect in the main pulmonary artery trunk (6/7) , left pulmonary artery (6/7) , right pulmonary artery (7/7).7 cases received TTE, showed enlarged right ventricle (6/7), which the mean right ventricular end-diastolic diamete was (38.54±16.30) mm; enlarged right atrium (7/7), which the mean right atrium diameter was (55.11±5.45) mm; and tricuspid insufficiency (7/7), which estimated mean pulmonary artery pressure was (81.14±21.17)mmHg, and pulmonary stenosis (5/7).4 cases received deep venous ultrasound, 1 of them had deep venous thrombosis (DVT).4 cases received Ganz catheter, the preoperative and postoperative mean pulmonary artery pressure was (52.00±5.23) mmHg and (23.00±5.60) mmHg, respectively.④9 patients receiving surgery, intimal sarcoma was diagnosed in all of them.Leiomyosarcomas was diagnosed in 3 case, leiomyo-angiosarcoma was diagnosed in 1 case, undifferentiated sarcomas was diagnosed in 1 case.⑤the 1-,3-and 5-month survival was 71.4%, 53.6% and 35.7%, respectively, survival median was 5 months.Conclusion While the patient with right atrium, right ventricular enlargement and right heart failure appeared earlier, and D-Dimer was in the normal range, PAS should be suspected.