Clinical features and surgical outcomes of pulmonary artery sarcoma

Abstract

Objective: Pulmonary artery sarcoma is an exceedingly rare malignancy. There is a lack of consensus regarding its diagnosis and treatment. We reviewed our experience in the surgical management of pulmonary artery sarcoma over an 11-year period.

Methods: From January 2005 to February 2016, 12 patients with pulmonary artery sarcoma (age 51 ± 14 years, 5 male) underwent surgical treatment at our center. Nine patients (75%) exhibited pulmonary trunk involvement on computed tomography angiography. Six patients (50%) were initially misdiagnosed with pulmonary embolism.

Results: Ten patients underwent pulmonary endarterectomy, and 3 of these patients required additional unilateral pneumonectomy. The remaining 2 patients underwent exploratory thoracotomy. One patient (8.3%) died in the hospital. The median length of postoperative intensive care unit and hospital stay were 1 day (range, 1-15 days) and 8 days (range, 5-21 days), respectively. The median postoperative survival of the total series was 18 months. Patients who received postoperative combined chemo- and radiotherapy were associated with improved survival compared with those who had isolated adjuvant therapy or surgery alone (median survival 28 vs 8 months, P = .042).

Conclusions: Although pulmonary artery sarcoma has a very poor prognosis, surgical treatment offers a chance for symptom relief and better long-term outcome. Aggressive postoperative adjuvant treatment may be necessary to improve survival.

Keywords: adjuvant therapy; outcomes; pulmonary artery sarcoma; survival.