肺原发性滑膜肉瘤临床病理观察  

Primary pulmonary synovial sarcoma: a clinicopathologic analysis of one case

作　　者：黄亚冰[1] 汤永飞[1] 胡健[2] 吴昊[1] 詹娜[1] 曾智[1] 阎红琳 王则胜[1] 

机构地区：[1]武汉大学人民医院病理科,武汉430060 [2]武汉大学人民医院放疗科,武汉430060

出　　处：《诊断病理学杂志》2016年第8期616-619,共4页Chinese Journal of Diagnostic Pathology

基　　金：国家自然科学基金重大研究计划培育项目(91130034)

摘　　要：目的探讨肺原发性滑膜肉瘤(PPSS)临床病理特点、诊断与鉴别诊断。方法分析1例PPSS临床资料,观察并检测其组织学形态、免疫组化特征及SYT-SSX基因易位情况,并复习相关文献。结果镜下肿瘤界限清晰,部分由梭形细胞构成。梭形成分形态较一致,核深染,有的形成腺管样腔隙。部分细胞呈圆形、卵圆形或立方形,胞质丰富、淡染,细胞界限清楚,核较大,空泡状,可见核仁。免疫组化:vimentin、EMA、CD99和bcl-2均(+),CK7、CK19、CK20、TTF1、PCK、CD117、Dog-1、S-100、CR、CD31、CD34、desmin和SMA均(-)。FISH检测示SYTSSX基因融合。病理诊断为肺原发性滑膜肉瘤(双相型)。结论肺滑膜肉瘤是一种罕见的肿瘤,应与肺梭形细胞癌、恶性间皮瘤、纤维肉瘤及恶性外周神经鞘膜瘤等疾病相鉴别。通过免疫组化方法并结合影像学资料,必要时进行分子遗传学检测可以明确诊断。Objective To explore the clinicopathological features, diagnosis and differential diagnosis of primary pulmonary synovial sarcoma （PPSS）. Methods One case of PPSS was studied by analysis of the clinical data, histological morphology and immunohistochemical characteristics, gene translocation of SYT-SSX and review of the literature. Results Microscopically, the tumor tissue showed a well boundary. In some area, the tumor cells were spindle with nucleus in same size and less mitosis. Some tumor tissue showed adenoid lacuna. In other area, the tumor cells were buninoid, ovate or cuboidal with abundant and hypochromatic cytoplasm. The cells showed obvious boundaries from other surrounding cells with bigger and vacuole nucleus, lmmunohistoehemical staining showed that tumor cells were positive for vimentin, EMA, CD99 and Bel-2, but negative for CK7, CK19, CK20, TFF1, PCK, CDII7, Dog-l, S-100, CR, CD31, CD34, desmin and SMA. Positive signals were confirmed by FISH. Pathological diagnosis was primary pulmonary synovial sarcoma （biphasic）. Conclusion The histopathologie diagnosis of primary pulmonary synovial sarcoma is challenging. It is often misdiagnosed as spindle cell carcinoma of lung, malignant mesothelioma, fibroma sarcomatosum or malignant peripheral nerve sheath tumor. However, based on the typical histopathologic features of this entity, definite diagnosis could be made by immunohistoehemieal reactions and radiological data, if necessary by molecular genetic analysis.

关 键 词：肺肿瘤 滑膜肉瘤 临床病理学 免疫组化 荧光原位杂交 

分 类 号：R734.21[医药卫生—肿瘤]