Clinical presentation and surgical treatment of primary pulmonary artery sarcoma.

OBJECTIVES: Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis modality and optimal surgical strategy remain unclear. The aim of this study was to report our surgical experience in this disease.

METHODS: Between 2000 and 2017, 13 patients underwent operations for PAS at our institution. Five patients underwent tumour resection alone, whereas the other 8 patients received pulmonary endarterectomy. The medical records were retrospectively reviewed to evaluate the clinical characteristics, operative findings and the postoperative outcomes.

RESULTS: There were 2 perioperative deaths, both had unresolved severe pulmonary hypertension. Follow-up was completed for all patients with a mean duration of 14.3 months (1-48 months). During follow-up, 6 patients died after a mean survival time of 20.2 months (4-48 months). For the 5 patients alive at follow-up (mean duration of 7.2 months), 3 already had local recurrence. For all 13 patients, the cumulative survival rates at 1, 2 and 3 years were 56.4, 37.6 and 18.8%, respectively. The 8 patients treated with pulmonary endarterectomy seemed to have a better survival rate compared with the remaining 5 patients without endarterectomy, although the difference was not statistically significant.

CONCLUSIONS: Primary PAS is often mistaken for chronic pulmonary thromboembolism. Surgical intervention is the mainstay of treatment for PAS, but the prognosis after surgery remains poor. Compared to isolated tumour resection, pulmonary endarterectomy seemed to yield a better survival rate.