| 梁靓,程玉芳.肺原发性滑膜肉瘤 4例临床病理及 SS18-SSX融合基因分析[J].安徽医药,2026,30(8):1587-1590. |
| 肺原发性滑膜肉瘤 4例临床病理及 SS18-SSX融合基因分析 |
| Clinical pathology and SS18-SSX fusion gene analysis of 4 cases of primary pulmonary synovial sarcoma |
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| DOI：10.3969/j.issn.1009-6469.2026.08.020 |
| 中文关键词: 肺肿瘤 肉瘤，滑膜 SS18-SSX融合基因 荧光原位杂交 免疫组织化学 |
| 英文关键词: Lung neoplasms Sarcoma, synovial SS18-SSX fusion gene Fluorescence in situ hybridization Immunohistochem-istry |
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| 中文摘要: |
| 目的探讨肺原发性滑膜肉瘤( PPSS)的临床特点、影像学检查和病理诊断特征及临床治疗。方法回顾性分析 2022年 12月至 2024年 9月阜阳市人民医院 4例病理诊断为 PPSS的临床表现、影像学、病理学特征、免疫组织化学及 SS18-SSX融合基因表型。结果 4例 PPSS病人的年龄范围为 51~75岁，均为男性，因咳嗽、胸痛、肺内结节等原因就诊，显微镜下形态 1例为单相纤维型滑膜肉瘤，肿瘤细胞表现为梭形一致呈束状排列， 3例为低分化型，显示大量梭形细胞混合不规则排列伴细胞异型、坏死。免疫组织化学表型:波形蛋白( vimentin)、转导样增强子结合蛋白 1(TLE1)、 B淋巴细胞瘤 -2蛋白( Bcl-2)、白细胞分化抗原 99(CD99)均为阳性， SS18-SSX部分阳性，荧光原位杂交( FISH)检测结果提示 3例 SS18融合基因。结论 PPSS在临床较为罕见，恶性程度较高，在形态学上可分为多种亚型，且存在 SS18-SSX融合基因变异，需结合 CT、病理、免疫组织化学、 FISH检测等综合诊断，临床治疗可采用手术切除辅助放化疗。 |
| 英文摘要: |
| Objective To explore the clinical characteristics, imaging findings, pathological diagnostic features, and clinical treat-ment of primary pulmonary synovial sarcoma (PPSS).Methods A retrospective analysis was conducted on the clinical manifestations,imaging, pathological features, immunohistochemistry, and SS18-SSX gene phenotype of 4 cases of PPSS diagnosed pathologically inFuyang People's Hospital from December 2022 to September 2024.Results The age range of the 4 PPSS patients was 51 to 75 years,all male. They presented with symptoms such as cough, chest pain, and pulmonary nodules. Microscopically, one case was monophasicfibrous synovial sarcoma, with tumor cells showing spindle-shaped cells arranged in bundles. Three cases were poorly differentiated,showing a large number of spindle cells mixed and irregularly arranged with cellular atypia and necrosis. Immunohistochemical pheno-types: vimentin, TLE1, Bcl-2, and CD99 proteins were all positive, and SS18-SSX was partially positive. FISH detection results indicat-ed SS18 fusion gene in 3 cases.Conclusions PPSS is relatively rare in clinical practice, with a high degree of malignancy. It can beclassified into multiple subtypes morphologically and has SS18-SSX fusion gene variations. A comprehensive diagnosis should be madeby combining CT, pathology, immunohistochemistry, and FISH detection. Clinical treatment can include surgical resection assisted byradiotherapy and chemotherapy. |
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