摘要目的 探讨儿童滑膜肉瘤的临床病理学特点及鉴别诊断.方法 收集2016至2018年北京市儿童医院诊断的12例儿童滑膜肉瘤,病变组织行HE染色、免疫组织化学标记和荧光原位杂交检测,观察其组织学形态、分子表达及遗传学情况.结果 双相型6例,单向上皮型1例,梭形细胞型3例,分化差型2例.上皮细胞膜抗原、广谱细胞角蛋白、bcl?2、CD99、TLE1阳性比例分别为10/12、9/12、12/12、10/12和10/12,CD34均为阴性,INI1表达呈特殊模式者占9/12.11例经FISH检测, SS18?SSX融合基因阳性比例为8/11.结论 滑膜肉瘤是儿童少见的具有双向分化特征的恶性软组织肿瘤,明确诊断需结合组织形态、免疫组织化学染色及分子病理特点综合考虑.

abstractsObjective To investigate histopathological characteristics, and differential diagnoses of childhood synovial sarcoma. Methods HE staining, immunohistochemical staining and fusion gene detection by FISH were performed in 12 cases of synovial sarcoma in childhood at Beijing Children′s Hospital from 2016 to 2018. Results There were 6 cases of biphasic type,1 case of monophasic epithelial type,3 cases of monophasic spindle cell type and 2 cases of poorly differentiated synovial sarcomas. EMA, CKpan, bcl?2, CD99, TLE1 and CD34 immunostain positivities were observed in 10/12, 9/12, 12/12, 10/12, 10/12 and 0/12 cases respectively. Unique INI1 immunohistochemical staining was observed in 9/12 cases. SS18?SSX gene fusion was detected in 8 of 11 cases by FISH.Conclusions Synovial sarcoma is rare in children. Histological morphology combined with immunohistochemistry and FISH SS18?SSX fusion gene detection are important for the diagnosis and differential diagnosis of synovial sarcoma in children.