Unusual Survival After Pulmonary Artery Sarcoma

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Abstract
A 46 yr old man with a primary sarcoma of the pulmonary artery is presented. The tumor was responsive to radiotherapy and the patient is the longest reported survivor of this rare disease. The tumor spread distally in the pulmonary vasculature, with probable embolization to the CNS. He died 27 mo. after the initial diagnosis, with sudden massive hemoptysis. Pulmonary artery sarcomas are a rare cause of obstruction of the right ventricular outflow. The course of this patient suggests that early recognition and therapy may provide some of these patients with effective palliation and prolonged survival.