| 崔芬芬,李元敏,宋 兵.肺动脉肉瘤诊断及治疗进展[J].肿瘤学杂志,2020,26(10):914-917. |
| 肺动脉肉瘤诊断及治疗进展 |
| Progress in Diagnosis and Treatment of Pulmonary Artery Sarcoma |
| 投稿时间：2019-08-08 |
| DOI：10.11735/j.issn.1671-170X.2020.10.B012 |
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| 中文关键词: 肺动脉肉瘤 肺栓塞 溶栓 肺动脉内膜剥脱术 肺切除术 |
| 英文关键词:pulmonary artery sarcoma pulmonary embolism thrombolytic pulmonary endarterectomy pneumonectomy |
| 基金项目:甘肃省自然科学基金（18JR3RA346） |
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| 中文摘要: |
| 摘 要：肺动脉肉瘤是一种极其罕见的肺血管系统恶性肿瘤，常起源于肺动脉内膜，其病因及发病机制不明。患者的临床表现、实验室及影像学检查常无明显特异性，故诊断难度大，常误诊为急慢性肺栓塞。CT肺动脉造影及MRI检查对肺动脉肉瘤的诊断有重要意义。当患者临床表现与影像学表现不符，溶栓治疗无效时，应高度警惕本病可能，其最终诊断需组织病理学检查。肺动脉内膜剥脱术、肺切除术等外科手术治疗可以改善患者的临床症状并延长患者的生存期，术后辅以放化疗可以增加存活率，但整体预后欠佳。 |
| 英文摘要: |
| Abstract：Pulmonary artery sarcoma(PAS) is an extremely rare malignancy of pulmonary vascular system. Its etiology and pathogenesis are unknown. The clinical manifestations，laboratory and imaging findings of the patients are often nonspecific. So the diagnosis is very difficult and it is easily misdiagnosed as acute or chronic pulmonary embolism. CT pulmonary angiography and MRI examination are of important value for the diagnosis of PSA. When the patient's clinical manifestations are not consistent with imaging findings，and thrombolytic therapy is ineffective，clinicians should be highly alert to the possibility of this disease. Final diagnosis of PSA requires histopathological examination. Although PAS has a very poor prognosis，pulmonary endarterectomy，pneumonectomy offer a chance for symptom relief and better long?鄄term outcome. Aggressive postoperative chemotherapy and radiotherapy may be necessary to improve survival of patients. |
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