ABSTRACT
We present a 62-year-old woman who was diagnosed with primary pulmonary arterial sarcoma with pulmonary hypertension. CT showed a large defect inside both main pulmonary arteries in accordance with the accumulation of FDG-PET. To relieve the symptom and to prevent sudden death, removal of a massive pulmonary tumor and postoperative chemotherapy were planned. Utilizing the intermittent systemic circulatory arrest under deep hypothermia (18°C), the pulmonary artery trunk and both main pulmonary arteries were opened. The tumor stacking inside the pulmonary artery was removed and its origin at the commissure of the pulmonary artery valve was resected. The defect was repaired with a pulmonary valve replacement. Histopathological examination revealed high grade sarcoma. Her postoperative course was uneventful ; however, she died of cerebral hemorrhage during chemotherapy six months after surgery.