Abstract: Objective To investigate the clinical features, differential diagnosis, surgical treatment and outcome of primary pulmonary artery sarcoma. Methods Between January 1994 and December 2004, 5 patients with primary pulmonary artery sarcoma were identified at operation and treated by surgical resection. Pulmonary valve stenosis were initially diagnosed in 3 patients, and chronic pulmonary embolism were initially diagnosed in 2 patients. Tumor resection from the vascular bed was performed in 1 patient and tumor resection and homograft reconstruction of pulmonary arteries were performed in 4 patients. Results One patient died of postoperative refractory pulmonary hypertension, 2 patients died 4 months after operation because of brain metastases, 1 patient was alive for 9 months after operation with recurrent pulmonary tumor, and 1 patient was alive for 2 years after operation without clinical or radiological signs of tumor recurrence or metastasis. Histological examinations showed 4 malignant mesenchymomas and 1 fibrosarcoma. Conclusions Primary pulmonary artery sarcomas are rare and usually fatal tumors of the cardiovascular system. The diagnosis is difficult and this disease is frequently misdiagnosed as chronic pulmonary hromboembolism and pulmonary valve stenosis. Early diagnosis can be improved by computerized tomography scanning and magnetic resonance imaging. Radi
cal surgical resection was the most effective modality for shortterm palliation. The prognosis of pulmonary artery sarcoma is poor. The survival time after resection varies from several months to several years depending on the presence of recurrence or metastasis.

Citation： HU Xiaopeng,FENG Jun,XU Jianping.. Diagnosis and Surgical Treatment of Primary Pulmonary Artery Tumor. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2007, 14(1): 23-26. doi: Copy