Outcome of Patients with Curative-Intent Treatment for Primary Pulmonary Sarcoma: Results of an International Multicenter Database

Background Primary pulmonary sarcoma (PPS) is a very rare entity accounting for less than 1% of all pulmonary malignancies. Data on PPS are scarce and rely mostly on small case series.Our aim was to evaluate the outcome and prognostic factors of patients with primary pulmonary sarcoma (PPS) who underwent curative-intent surgery within a multimodality treatment framework.

Methods & Materials An international multicenter database of patients with PPS was created through a network of sarcoma experts. Data on demographics, staging work-up, multimodality treatment including surgery, and outcomes were retrieved. Overall survival was calculated from the date of diagnosis. Prognostic factors were assessed using uni- and multivariate analysis.

Results Eighteen centers from nine countries contributed to the database, with a total of 173 patients. One hundred fifteen patients (66%) underwent curative-intent surgery within multimodality treatment. Most had symptoms at diagnosis (n=88, 77%). There were 58 males (50%). A history of smoking was present in 32 patients (28%). Metastases were observed in 23 patients (20%), mainly lung metastases (n=7, 30%) or pleural sarcomatosis (n=9, 39%). Preoperative chemotherapy was administered to 33 patients (30%). The extent of lung resection was sublobar (n=11, 10%), lobar (n=58, 50%), or bilobar/pneumonectomy (n=39, 34%). Synovial sarcoma was the most common histologic type (n=49, 44%). The median tumor size was 85 mm. Sixty-nine patients had grade 3 tumors (72%). Resection was complete (R0) in 85 patients (74%). Lymphadenectomy was performed in 70 patients (63%), with nodal involvement in 10 patients (14%). Adjuvant chemotherapy was administered as in 37 (37%) patients, while 27 patients received adjuvant radiotherapy (27%) patients, respectively. Overall survival was 49% and 31% at five and ten years, respectively. The median follow-up period was 33 months. Male gender (p=0.003), age over 60 years (p=0.021), presence of metastasis (p=0.002), tumor size larger than 40 mm (p=0.046), and incomplete resections (p=0.008) were independent prognostic factors for poor survival.

Conclusion In patients with curative-intent multimodal treatment for PPS, an encouraging 5-year survival rate of 49% can be achieved in expert centers. Independent prognostic factors may aid in selecting patients for curative treatment.

 

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Publication History

Article published online:
25 August 2025