With surgical examination, the pulmonary artery may be nonpulsative, firm and noncompressible. The intrapericardium must be explored to evaluate the proximal extent of the tumor. If lung resection is an option, the main bronchus and superior and inferior pulmonary veins are transected. The pulmonary artery is transected at a point proximal to the origin of the tumor. Cardiopulmonary bypass may be necessary to gain control of the proximal pulmonary artery.
Patients receiving chemotherapy and radiotherapy alone have a poorer prognosis.
Long term survivors have typically been treated with surgical excision, with or without additional therapy. Primary Pulmonary Artery Sarcoma-A Report of Two Cases, with Special Emphasis on the Diagnostic Problems. Delaney SG, TC Doyle, RW Bunton, NA Hung, LU Joblin and DR Taylor. Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism. Hiroshima K, T Uruma, M Ishibashi, H Ohwada and Y Hayashi.