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原发性肺滑膜肉瘤2例并文献复习

  

  1. 中山大学孙逸仙纪念医院 呼吸与危重症医学科,广东 广州 510120
  • 出版日期:2019-03-20 发布日期:2019-04-08
  • 通讯作者: 通信作者:李建国, Email:jgli88@126.com
  • 基金资助:
    广州市科技计划项目---骨髓间充质干细胞外泌体miR-1470调控哮喘调节性T细胞功能的机制研究(201604020103)

Primary pulmonary synovial sarcoma: report of 2 cases and literature review

  1. Department of Respiratory and Critical Care Medicine, Sun Yansen Memorial Hospital, Sun Yansen University, Guangzhou 510120, China
  • Online:2019-03-20 Published:2019-04-08
  • Contact: Corresponding author: Li Jianguo, Email:jgli88@126.com

摘要: 回顾分析2例原发性肺滑膜肉瘤(PPSS)患者, 病程均较短,胸部CT示:肺部肿块,边界清楚,密度分布不均匀,增强扫描呈不均匀强化。病理结果:可见密集增生的梭形细胞,胞浆少,核浆比升高,染色质细腻,核仁不明显,可见核分裂像。免疫组化:上皮细胞膜抗原、波形蛋白、CD99、细胞角蛋白(+),CD34、Actin、S100、Desmin(-),Ki67局部(+)。基因检测:SS18基因断裂检测(FISH法)阳性。结合胸部CT、病理、免疫组化及融合基因检查可提高对PPSS的诊断水平。

关键词: 肉瘤,  , 滑膜 , 肺, 胸部CT, 病理特征, 免疫组织化学, 融合基因

Abstract: A retrospective study was performed in 2 cases of primary pulmonary synovial sarcoma (PPSS) with short course of the disease. Chest CT scan showed that the lung mass had the characteristics of definite boundaries, uneven density distribution, and heterogeneous  enhancement on CT  enhanced scan. Pathological examination showed the dense proliferation of spindle cells with less cytoplasm, higher nucleoplasmic ratio, fine chromatin, inconspicuous nucleoli, and mitotic figure. Immunohistochemistry revealed that the cells were positive for epithelial membrane antigen(EMA), vimentin, CD99 and CK, negative for CD34,  Actin,  S100 and Desmin, and partially positive for Ki67. Cytogenetics showed SS18 gene fragmentation test (FISH method) was positive. Conclusively, the diagnostic level of PPSS can be improved with the combination of  chest  CT sacn, pathological examination, immunohistochemistry and the examination of fusion gene.

Key words: sarcoma, synovial, lung; , chest ct scan; , pathological features, immunohistochemistry, fusion gene