Primary Intrathoracic Synovial Sarcoma: An Analysis of Outcomes of This Rare Disease

Background: Primary intrathoracic synovial sarcoma (SS) is a rare entity. The objective of this study was to evaluate survival outcomes for patients with intrathoracic SS presenting with localized disease at diagnosis. Methods: We conducted a retrospective review of 63 patients diagnosed with intrathoracic SS between 1997 and 2020. The Kaplan-Meier method and log-rank test were used to estimate the progression-free survival (PFS), overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS). The hazard ratios were estimated by using Cox proportional hazards regression. Median follow-up time, age-at-diagnosis, and primary tumor size were 31 months (range: 4-218 months), 43 years (range: 18-77), and 7 cm (range: 1-23), respectively. Results: Sixty-two of sixty-three (98%) patients had their primary tumor resected, from whom eighteen (29%) and forty-three (69%) had received neo/adjuvant radiotherapy and chemotherapy, respectively. Median PFS, OS, and MFS were 1.2, 3.0, and 1.1 years, respectively. Based on multivariable analyses, patients with ≥5 cm tumor size had poorer OS (versus < 5 cm; HR: 2.66; 95% CI: 1.16, 6.11; LR-p = 0.014). Importantly, the receipt of neo/adjuvant chemotherapy was the only factor associated with both a more favorable PFS (HR: 0.33; 95% CI: 0.17, 0.65; LR-p = 0.0002) and a more favorable MFS (median 1.33 years versus no chemo 0.5 years; HR: 0.35; 95% CI: 0.17, 0.73; LR-p = 0.005). Conclusions: Outcomes associated with intrathoracic SS remain poor. Factors associated with poorer outcomes include larger tumors and omission of chemotherapy in the management of localized disease. We recommend providing perioperative chemotherapy to all patients with ≥5 cm tumor size to improve progression and metastasis-free survival.