肺滑膜肉瘤的诊治现状

Diagnosis and treatment of pulmonary synovial sarcoma

目的探讨提高肺滑膜肉瘤诊断、治愈率的方法。方法选择1例肺滑膜肉瘤患者，同时结合2012年10月中国期刊全文数据库收录发表的80例资料较为完整的肺原发性滑膜肉瘤患者进行回顾性研究，分析其主要临床表现、影像学特点，根据病理学、免疫组化特点及分子生物学对其进行诊断及鉴别诊断，以减少误诊。结果81例肺原发性滑膜肉瘤患者中，男40例，女41例。主要临床表现有胸痛、呼吸困难、咯血及咳嗽。个别病例无任何不适。全部病例均进行手术治疗。术前均未确诊，最终依赖病理、免疫组化支持诊断。结论肺原发性滑膜肉瘤临床表现不一，影像学检查具有相对特征表现，易与其他肺部肿瘤或炎性反应混淆；确诊需依靠病理、免疫组化和融合基因检测。手术切除是主要的治疗方法，切除不完全者预后差。

Objective To investigate the way of improving the diagnosis and cure rate of pulmonary synovial sarcoma. Methods One case of pulmonary synovial sarcoma was selected, at the same time accompanied with 80 cases of pulmonary synovial sarcoma with complete data referenced from China Journal Full-text Database on October 2012 were retrospectively studied. The main clinical manifestations,imaging characteristics were analyszed. According to the pathology, immunohisochemistry and molecular biology for diagnosis and discrimination, in order to reduce the misdiagnosis. Results All of 81 patients, 40 male cases and 41 female cases,the main clinical manifestations were chest pain, difficult breathing, hemoptysis and cough. In individual cases without any discomfort. All cases had not been accurately diagnosed before surgery. Ultimately diagnosis of patients depend on histopathology and immunohistochemistry. Conclusions Pulmonary synovial sarcoma shows different clinical symptoms but specific imaging characteristic and maybe confused with other pulmonary tumors or inflammation. Histopathological, immunophenotypic and fusion gene detection is the mainly technics for diagnosis. Surgery is the primary therapy,patients undergoing uncompletely resection have a poor prognosis.