Background: Pulmonary sarcoma arising from the pulmonary artery is a rare disease and its prognosis is disastrous.
Methods: Five patients who underwent surgery for pulmonary artery sarcoma were reviewed.
Results: All patients except one were initially diagnosed with pulmonary embolism. One patient with preoperative profound shock could not wean from cardiopulmonary bypass. Two patients are still surviving for 36 and 30 months, respectively.
Conclusions: Early diagnosis and complete surgical resection is perhaps the best way to improve patients with pulmonary artery sarcoma.