Sarcoma da Artéria Pulmonar — dificuldades diagnósticas e terapêuticas

ABSTRACTPrimary 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 7 th day after surgery. Pathology revealed pulmonary artery sarcoma with pulmonary and pleural metastases.REV PORT PNEUMOL IX (1): 41-5