Abstract
Primary Sarcoma of the Pulmonary Artery (PAS) is a very rare and miserable disease. The clinical signs and symptoms of PAS are non- specific, which usually prevents diagnosis before surgery or autopsy. The current guidelines for the diagnosis and treatment of PAS have not been well established. Several reported cases of PAS have been mistaken for Pulmonary Artery Thromboembolism (PTE), because the clinical signs and symptoms of PAS are non-specific. Elevated coagulation markers are generally absent in PAS and therefore, support a differential diagnosis of PTE. We herein report a patient with PAS who presented with elevated coagulation markers and later showed rapidly improved values mimicking response of PTE to anticoagulant therapy.