Haj Khalaf M, Higaze M, Kikoyan H, Hartmann A, Agaimy A, Stöhr R, Rieker R, Sirbu H (2026)
Publication Type: Journal article, Review article
Publication year: 2026
Book Volume: 21
Article Number: 341
Journal Issue: 1
DOI: 10.1186/s13019-026-04296-2
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.
APA:
Haj Khalaf, M., Higaze, M., Kikoyan, H., Hartmann, A., Agaimy, A., Stöhr, R.,... Sirbu, H. (2026). A rare case of relapsed primary pulmonary synovial sarcoma (PPSS) following surgery with multidisciplinary team management: case report and systematic review of literature. Journal of Cardiothoracic Surgery, 21(1). https://doi.org/10.1186/s13019-026-04296-2
MLA:
Haj Khalaf, Mohamed, et al. "A rare case of relapsed primary pulmonary synovial sarcoma (PPSS) following surgery with multidisciplinary team management: case report and systematic review of literature." Journal of Cardiothoracic Surgery 21.1 (2026).
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