Surgical treatment of pulmonary artery sarcoma: a report of 17cases

摘要

Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosisis often delayed, and optimal treatment remains unclear. The aim of this studyis to report our experience in the surgical management of this disease. Between2000 and 2018, 17 patients underwent operations for PAS at our center. Themedical records were retrospectively reviewed to evaluate the clinicalcharacteristics, operative findings, the postoperative outcomes, and thelong-term results. The mean age at operation was 46.0 ± 12.4 years (range, 26–79years), and eight (47.1%) patients were male. Six patients underwent tumorresection alone, whereas the other 11 patients received pulmonary endarterectomy(PEA). There were two perioperative deaths. Follow-up was completed for allpatients with a mean duration of 23.5 ± 17.6 months (1–52 months). For all 17patients, the median postoperative survival was 36 months, and estimatedcumulative survival rates at 1, 2, 3, and 4 years were 60.0%, 51.4%, 42.9%, and21.4%, respectively. The mean survival was 37.0 months after PEA and 14.6 monthsafter tumor resection only (p = 0.046). Patients who had nopulmonary hypertension (PH) postoperatively were associated with improved mediansurvival (48 vs. 5 months, p = 0.023). In conclusion, PAS isoften mistaken for chronic pulmonary thromboembolism. The prognosis of this veryinfrequent disease remains poor. Early detection is essential for prompt andbest surgical approach, superior to tumor resection alone, and PEA surgery withPH relieved can provide better chance of survival.