Abstract
Pulmonary artery sarcoma (PAS) is a rare, aggressive cancer originating from the intimal layer of the pulmonary artery (PA), often mistaken for pulmonary thromboembolism. This case report underscores the complex management of PAS and the necessity of a multidisciplinary approach for accurate diagnosis and treatment. A 52-year-old woman with PAS was diagnosed using imaging and therapeutic tests to distinguish it from pulmonary embolism. Primary treatment included surgical resection of the pulmonary trunk, valve, and tumor, followed by reconstruction. Complete resection was impossible due to extensive endocardial infiltration in the right ventricle, precluding cardiac transplant. The patient underwent adjuvant radiotherapy; however, the disease recurred, and she died 3 years post-diagnosis. This case highlights the rarity of an extensive right ventricle invasion, the absence of clear PAS management guidelines, and the limited evidence on the effectiveness of adjuvant therapies. It concludes that multidisciplinary teams are vital for decision-making and stresses the need for further research to establish effective treatment protocols.