RESUMEN
Primary Pulmonary Artery Sarcoma is a rare entity, which shares some clinical features with Thromboembolic Pulmonary Disease (TEPD), complicating differential diagnosis. The authors report a Clinical Case of a Primary Pulmonary Artery Sarcoma in a 59 years old man, admitted with a history of dyspnoea on exertion, chest pain and general symptoms. Chest X-ray, Computed Tomography Scan, Angiographies and Magnetic Resonance Imaging suggested TEPD. Blood Analysis performed before anticoagulation therapy: Lupus Anticoagulant-and Ig M Anticardiolipin +. Our presumptive initial diagnosis was TEPD in a patient with a hypercoagulable state. Intravenous heparin was started, with some clinical improvement but 2 months later he was readmitted, due to clinical and radiological deterioration. Pulmonary Thromboendarterectomy was considered but a right pneumonectomy was necessary because of bleeding. He died of ARDS in a single lung in the 7th day after surgery. Pathology revealed pulmonary artery sarcoma with pulmonary and pleural metastases.