PULS (Pulmonary Artery Sarcoma) – An International Collaboration for the Assessment of Pulmonary Artery Sarcoma Patients’ Outcome
Bibliographic record
Abstract
Abstract Background Pulmonary artery sarcomas (PAS) are rare with a poor prognosis and limited curative therapeutic options. This scarcity of evidence poses significant challenges. Aims This study aims to enhance the understanding of clinical characteristics and current management of PAS. Methods An international working group (PULS) was launched. Patients with PAS treated at participating institutions between 1994-2024 were included. Six centers collected data from patients diagnosed with PAS retrospectively. Results Among 49 patients with PAS, the median age was 54.0 years (range: 27.0–82.0), including 24 females (49.0%). Chest pain, dyspnea, and cough were present in 24.5% (n=12), 42.9% (n=21), and 24.5% (n=12), respectively. Initial diagnosis included PAS in 26.5% (n=13) and embolism in 24.5% (n=12). PAS was confirmed by surgery in 40.8% (n=20), imaging in 16.3% (n=8), and biopsy in 12.2% (n=6) and this information is missing in 30.6% (n=15). 33 (67.3%) patients underwent surgery. Tumor endarterectomy was most common, (n=20, 40%), including 1 (2%) patient undergoing additional lobectomy, and two (4.1%) patients additional pneumonectomy. 9 (18.3%) patients underwent pneumonectomy only and 4 (8.2%) debulking surgery only. 10 (20.4%) patients underwent multimodal treatment. One (2%) patient received neoadjuvant systemic therapy. Postoperative adjuvant systemic therapy (Doxorubicin/Ifosfamide) was given to 7 patients (14.3%) and 6 patients (12.2%) underwent radiotherapy. The 1-,2- and 5-year survival was n=20 (40%), n=14 (28.5%), and n=1 (2%) respectively. The subgroup of patients undergoing multimodal treatment had a better overall survival (p=0.05). Relapse of PAS occurred in 32.7% (n=16). Conclusion To date this is one of the largest reported multi-center cohort of patients with PAS. Our findings highlight the clinical variability in PAS presentation, challenges in achieving a timely diagnosis, and poor prognosis. Outcomes are most promising if patients undergo surgery embedded in multimodality treatment compared to patients undergoing surgery alone.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".