Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma

The study aimed to determine the optimal surgical procedure to treat pulmonary artery sarcomas responsible for pulmonary hypertension.

Between 1997 and 2010, 31 patients were treated surgically for pulmonary artery sarcomas. Sixteen patients were male; the mean age was 56 years (range, 26-78 years). Common symptoms were characteristic of acute or chronic pulmonary thromboembolic disease. Also, 21 patients experienced mild to severe pulmonary hypertension, with a mean total peripheral resistance of 473 dyn s cm(-5). Clinical presentation and preoperative work-up confirmed the suspicion of pulmonary artery sarcoma in 18 patients. The required surgical procedures included the following: pulmonary endarterectomy in 25 patients (combined with a right pneumonectomy in five and with a replacement of the main pulmonary artery by a homograft reconstruction in one), pneumonectomy only in five (three right and two left), with the use of cardiopulmonary bypass in three cases. In one patient, the right pulmonary artery only was replaced on cardiopulmonary bypass.

Final pathology showed 26 high-grade and five intermediate-grade sarcomas. The 30-day mortality was 13% (four patients). Repeat pulmonary resection was required in two patients due to recurrent disease. Moreover, 18 patients received adjuvant therapy. Mean follow-up was 19 months (range, 1-99 months); of the 11 patients alive at follow-up, four were noted to have recurrent disease. The 1-, 3- and 5- year survival was 63, 29 and 22%, respectively.

The prognosis of this very infrequent disease remains poor. Bilateral pulmonary endarterectomy may yield significant survival rates because it provides completeness of resection without sacrificing the pulmonary vascular bed.