BACKGROUNDPulmonary artery sarcoma originating from the pulmonary artery is a rare disease and its prognosis is extremely poor. Most patients are initially thought to have pulmonary embolism.

METHODSFive patients (3 males and 2 females, mean age was (42.0 ± 8.5) years) of primary pulmonary artery sarcomas underwent surgical procedures. Sarcoma resection and right ventricle outflow tract reconstruction were performed.

RESULTSThere was no death 30 days post-operation except one patient. The function of heart was improved to NYHA II.

CONCLUSIONSGiven the significant morbidity and mortality of pulmonary artery sarcomas, an aggressive strategy for resection is indicated. Early diagnosis and complete surgical resection is perhaps the best way to improve patients' survival with pulmonary artery sarcoma.