OBJECTIVETo investigate the clinical characteristics of pulmonary artery sarcoma (PAS) and to improve doctors' awareness and the early diagnosis of this disease.

METHODSThe 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-Dimer 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 TTE 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. Leiomyosarcoma was diagnosed in 3 cases, leiomyo-angiosarcoma was diagnosed in 1 case, undifferentiated sarcomas was diagnosed in 1 case. (5)The 1-, 3- and 5-month survival was 71.4%, 53.6% and 35.7%, respectively, median survival time was 5 months post surgery.

CONCLUSIONPAS is difficult to differentiate with PTE. PAS should be suspected in patient with right atrium, right ventricular enlargement and early appeared right heart failure, and normal D-Dimer level. Outcome for PAS patients is poor despite surgery in this cohort.