Pulmonary Artery Sarcoma

This type of pulmonary vascular tumor differs primarily from epithelioid hemangioendothelioma and angiosarcoma on account of its intravascular location and fibroblastic or leiomyomatous nature. Clinically, pulmonary artery sarcoma may present as a single large pulmonary embolus or multiple smaller emboli. Some may present with symptoms and signs right sided cardiac failure. CT tomography, MRI and angiography are of great value in the detection of these tumors but even with these technologies a premortem diagnosis is only made in half of the cases. Intraluminal masses can be detected by pulmonary angiography showing smooth tapering of pulmonary arteries and “to and fro” motion of pedunculated or lobulated tumors. By virtue of their intravascular location, endoscopy does not contribute much to the visualization of these tumors unless they have directly spread in the lumen of the airways.