A Case of Primary Pulmonary Artery Liposarcoma

Kang, Kyung Tae; Jeong, Myung Ho; Ahn, Young Keun; Cho, Jeong Gwan; Park, Chang Soo; Park, Jong Chun; Kang, Jung Chaee

Abstract

Primary pulmonary artery liposarcoma is one of rare tumors originating from pulmonary artery and mimicking pulmonary thromboembolism. The prognosis of primary pulmonary artery sarcoma is poor and we hereby report one case of pathologically proven pulmonary artery liposarcoma. A 57-year-old man presented with progressive dyspnea on exertion for 1 month. Jugular venous pressure was elevated up to 15 cm blood and increased P2 with systolic ejection murmur was heard. Initially, pulmonary thromboembolism was suspected by the signs of right heart failure and multiple perfusion defects on lung perfusion scan. Pulmonary arteriogram and cardiac MRI revealed huge mass obstructing right main pulmonary artery and the mass was partially excised for the improvement of pulmonary arterial blood flow. Tumor cells disclosed vimentin positive cells with abundant vacuolated cytoplasm, which were filled with lipid droplets by electron microscopic examination. Adjunctive radiotherapy was performed after surgery, but the patient died 6 months after discharge.