A Case of Pulmonary Artery Intimal Sarcoma Successfully Treated by Left Pneumonectomy and Artificial Blood Vessel under Cardiopulmonary Bypass

Abstract

Pulmonary artery intimal sarcoma is a rare disease associated with poor prognosis. The incidence of orphan disease is 0.001–0.03%, but it must be underestimated because pulmonary sarcoma frequently mimics pulmonary vascular disease, such as acute pulmonary embolism, chronic thromboembolic pulmonary hypertension. Clinical presentations are unusually with symptoms of cardiorespiratory dysfunction, and although early diagnosis is always difficult. We reported the case of male who was admitted to our hospital with abnormal chest shadow. A computed tomography (CT) scan showed a tumor invaded the main pulmonary artery from the left pulmonary artery. The tumor was aggressively resected with left pneumonectomy and reconstruction of the main pulmonary artery to right pulmonary artery using an artificial blood vessel under cardiopulmonary bypass. Although surgical resection is the standard of care, radical complete resection is rarely reported for tumors involving the main PA. We introduced a heart-lung machine under local anesthesia before introducing general anesthesia, and started the heart-lung machine at the same time as the start of surgery. Hemodynamics were stable and surgery could be performed safely. Complete tumor resection was possible by revascularization using artificial blood vessels. As a result, no local recurrence was observed 6 months after the operation, and a prognosis of 9 months was obtained.