Surgical treatment for pulmonary artery sarcoma

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Pulmonary artery sarcomas are exceedingly rare and the prognosis for patients with pulmonary artery sarcoma is very poor. We retrospectively reviewed the early and late outcomes after treatment for pulmonary artery sarcoma, and the purpose of this study is to report our surgical experience with this fatal disease. Between 1999 and 2007, a total of nine patients (mean age, 47.4 years; M:F = 4:5) underwent operations for pulmonary artery sarcoma at our institution. The tumor was radically resected and every effort was made to remove the tumor as completely as possible. Seven patients underwent surgical resection with the aid of hypothermic cardiopulmonary bypass. The completeness of resection was determined intraoperatively by frozen section biopsy of the resection margin. There was no in-hospital mortality. No patients suffered from significant complications related to the operation. Follow-up was completed for all the patients with a mean duration of 19.2 months. During follow-up, six patients died with a median survival time of 17.6 months. The cause of death was related to the recurrence of pulmonary artery sarcoma in all cases. The pattern of recurrence was local recurrence and distant metastasis in three and four patients, respectively. The early outcomes after surgical treatment for pulmonary artery sarcoma were excellent, and the late outcomes in this series were no worse than those in the previous reports. We suggest that the use of cardiopulmonary bypass is important to obtain a complete resection and the completeness of the resection should be confirmed intraoperatively by frozen section biopsy of the resection margin.