A systematic review of 146 primary pulmonary synovial sarcoma cases found that 91.1% underwent surgical resection, 51.4% received chemotherapy, and 24.0% received radiotherapy.
Systematic Review (n=146)
Primary pulmonary synovial sarcoma is a rare malignancy primarily treated with surgical resection, though recurrence remains a major challenge requiring multidisciplinary management.
Abstract Background Primary pulmonary synovial sarcoma (PPSS) is a rare and invasive subtype of soft tissue sarcoma that originates in the lung. Because of its rare incidence, nonspecific presentation, and radiographic overlap with other thoracic malignancies, diagnosis is often delayed. This systematic review aims to synthesize evidence to improve clinical management of such case presentations. Methods This systematic review was conducted and reported in accordance with the PRISMA statement, which guided the literature search strategy, data extraction, and data management. To ensure structured and consistent data collection, the PICO framework was applied to define the population, intervention, comparison, and relevant outcomes for each individual reported case of primary pulmonary synovial sarcoma (PPSS). Methodological quality of the included reports was assessed using the Murad tool for case reports and case series. The search targeted single-case reports and case series published through April 2025. In addition, we report a 58-year-old male patient with a confirmed primary pulmonary synovial sarcoma (PPSS). Treatment was provided by a multidisciplinary team (MDT) at the Department of Thoracic Surgery, University Hospital Erlangen. Results The result of our systematic review revealed a total of 146 PPSS cases. Geographical distribution: Asia (56.6%, 82 cases), the Americas (21.4%, 31), Europe (20%, 29), and Africa (2%, 4). The majority of reported patients were male (61.6%, 90), and (29.5%, 43) reported smoking. The surgical resection was performed in (91.1%, 133), chemotherapy in (51.4%, 75), and radiotherapy in (24.0%, 35). Most patients underwent open surgical resection (72.6%), predominantly lobectomy (65.1%), reflecting advanced tumor size at diagnosis, with 80.1% of tumors measuring ≥ 3 cm. The monophasic subtype was the most common (62.3%, 91), followed by the biphasic subtype (22.0%, 32) and the poorly differentiated subtype (15.8%, 23). At the time of publication, the reported patient shows no evidence of disease (NED). Conclusion PPSS requires early diagnosis and multidisciplinary management. Surgical resection is still the most important measure, supplemented by chemotherapy and/or radiotherapy. However, recurrence remains a major challenge. Further research is needed to standardize treatment and improve outcomes.
Khalaf et al. (Thu,) conducted a systematic review in Primary pulmonary synovial sarcoma (PPSS) (n=146). Multidisciplinary management (surgical resection, chemotherapy, radiotherapy) was evaluated on Treatment modalities utilized. A systematic review of 146 primary pulmonary synovial sarcoma cases found that 91.1% underwent surgical resection, 51.4% received chemotherapy, and 24.0% received radiotherapy.
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