原发性肺动脉肿瘤的诊断与外科治疗

Diagnosis and Surgical Treatment of Primary Pulmonary Artery Tumor

目的探讨原发性肺动脉肿瘤的诊断和外科治疗,提高对原发性肺动脉肿瘤的认识。方法1994年1月至2004年12月,我院收治的5例患者经手术和病理检查证实为原发性肺动脉肿瘤。术前误诊为肺动脉瓣狭窄3例,肺动脉血栓栓塞2例。行单纯肺动脉肿瘤切除术1例,肺动脉肿瘤切除+同种肺动脉置换术4例,同期行右心室流出道加宽补片2例。结果手术死亡1例;2例术后4个月死于脑转移;1例术后存活9个月,局部复发;1例术后存活2年,无肿瘤复发和转移。病理诊断:肺动脉恶性间叶细胞瘤4例,肺动脉纤维肉瘤1例。结论原发性肺动脉肿瘤非常少见,临床表现与肺动脉血栓栓塞等肺动脉阻塞性疾病并无明显差异,诊断困难。电子束CT和磁共振成像有助于该病的诊断。治疗的关键是外科手术彻底切除肿瘤并结合放疗和化疗,但易发生局部复发和转移,预后较差。

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 thromboembolism and pulmonary valve stenosis. Early diagnosis can be improved by computerized tomography scanning and magnetic resonance imaging. Radical 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.