Primary pulmonary synovial sarcoma.

Objective: Primary pulmonary synovial sarcoma (PPSS) is extremely rare. This study aims to identify the clinicopathologic and therapeutic factors determining survival in PPSS.

Methods: We performed a retrospective analysis of 121 patients from the Surveillance, Epidemiology, and End Results Database as well as 12 patients from our own institution diagnosed with PPSS. Patient survival was evaluated using the Kaplan-Meier method.

Results: The median survival time for 12 PPSS patients in our institution was 78 months. Postoperative chemotherapy ( = .027 for overall survival and  = .035 for disease-specific survival) was associated with superior survival, whereas pneumonectomy ( = .011 for overall survival and  = .006 for disease-specific survival) was associated with worse survival. Single lobe involvement ( = .022) and the absence of lymph node involvement ( = .045) were associated with improved disease-specific survival and overall survival, respectively. In the Surveillance, Epidemiology, and End Results Database, the median survival time was 23 months. Significantly superior survival was observed in patients with earlier American Joint Committee on Cancer stage (Ⅰ-Ⅱ) ( < .001 for both overall survival and disease-specific survival). Patients who were diagnosed within the recent decade did not achieve a better survival ( = .599 for overall survival and  = .596 for disease-specific survival).

Conclusions: PPSS was aggressive with a very poor prognosis. The seventh American Joint Committee on Cancer stage might aid in predicting survival. Pneumonectomy and lymph node involvement might be associated with worse survival, whereas single lobe involvement and postoperative chemotherapy might be associated with improved survival.