Clinical Features of Primary Pulmonary Artery Sarcoma: A Systematic Review and Pooled Analysis.

Objectives: Primary pulmonary artery sarcoma (PPAS) is a rare disease with unclear clinical manifestations. Advances in imaging devices have improved diagnostic capabilities, potentially affecting clinical characteristics and overall survival (OS); however, details remain unclear. This study conducted a pooled analysis of case reports and series to analyse the clinical characteristics and OS of PPAS in the era of advanced medical devices.

Methods: Data were sourced from PubMed and CINAHL, focusing on studies published between 1 January 2014 and 31 December 2023. The study included patients diagnosed with PPAS, with extracted data covering demographics, diagnosis, treatments, and survival.

Results: Overall, 643 patients were included (mean age: 52.6±13.1 years; 50.4% were female). Initially, 70.6% were diagnosed with pulmonary thromboembolism (PTE), and 15.4% were suspected of having PPAS. Among these, 93.9% and 55.2% showed suggestive findings on computed tomography (CT)-integrated positron emission tomography with 2-deoxy-2-18F-fluoro-d-glucose (18F-FDG PET/CT) and CT, respectively, with 98.2% confirmed before death. The right main pulmonary artery was the most affected site on CT (72.3%). Surgery and chemotherapy were performed in 81.4% and 66.4% of patients, respectively. The median OS was 31 months, with surgery extending OS across all stages and chemotherapy benefiting stages III-IV. Longer OS was achieved in patients who underwent complete surgical resection.

Conclusions: 18F-FDG PET/CT and multi-detector-row CT can differentiate PTE from PPAS. These medical devices may contribute to improved OS.