Primary Pulmonary Artery Intimal Sarcoma Case with Elevated Coagulation Markers

• Main Authors: Koshiro Sakai, Yoshino Minoura, Taiju Matsui, Kyoichi Kaneko, Yoichi Kobayashi
• Format: Article
• Language: English
• Published: JCDR Research and Publications Private Limited 2017-04-01
• Series: Journal of Clinical and Diagnostic Research
• Subjects: d-dimer; hypercoagulability; super-hyperfibrinolysis
• Online Access: https://jcdr.net/articles/PDF/9609/23423_CE(EK)_F(RK)_PF1(P_RK)_PFA(P)_PF2(ShGH).pdf

Primary Sarcoma of the Pulmonary Artery (PAS) is a very rare and miserable disease. The clinical signs and symptoms of PAS are nonspecific, 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.