Abstract
Pulmonary artery intimal sarcoma (PAS) is an exceedingly rare malignant tumor that frequently mimics pulmonary embolism, leading to delays in diagnosis and treatment. We report the case of a 41-year-old woman who presented with progressive dyspnea and was initially treated for presumed pulmonary embolism. Advanced imaging revealed features suggestive of a primary pulmonary artery tumor, and she underwent surgical resection, confirming high-grade intimal sarcoma. Despite recurrence four years later requiring repeat surgery and eventual metastatic progression, the patient survived more than five years, substantially longer than typical outcomes reported for this malignancy. This case highlights the importance of early recognition, the diagnostic value of multimodality imaging, and the critical role of aggressive surgical management in improving survival in PAS.