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Objective: Primary pulmonary artery sarcoma is a severe and underdiagnosed disease, with the clinical and surgical approach not clearly established. Only a few individual case reports or small series on this topic have been published. The aim of the present study was to report our surgical experience in this field., Methods: From March 2004 to December 2012, 13 patients underwent surgery for pulmonary artery sarcoma at our institution. In 7 patients, the sarcoma was unilateral (53.8%), and in 6 (46.2%), the tumor had already extended to both lungs. The surgical strategy evolved over the years, but the 2 techniques used were always the same: pneumonectomy in 5 patients and pulmonary endarterectomy in 8., Results: Two patients died in-hospital, both in the pneumonectomy group. The median length of the intensive care unit and hospital stay was 1 day (range, 1-10) and 14 days (range, 11-17) for the pneumonectomy group and 6 days (range, 3-23) and 19 days (range, 10-32) fort the pulmonary endarterectomy group, respectively. The median survival was 26.8 months after pneumonectomy and 6.6 months after pulmonary endarterectomy., Conclusions: Primary pulmonary artery sarcoma has a poor prognosis. The surgical strategy at our institution included pneumonectomy, for possible radical resection, and palliative endarterectomy, to reduce symptoms and increase the life expectancy. The correct surgical approach must be evaluated individually, according to the tumor presentation, the presence of pulmonary hypertension, and the patient's clinical condition., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)