儿童梭形细胞型滑膜肉瘤的临床病理特征分析  

Analysis of Clinicopathological Featurs of Spindle Cell Type Synovial Sarcoma in Childhood

作　　者：熊枫[1] 黄慧[1] 樊金星 杨文萍[1] 吴艳[1] XIONG Feng;HUANG Hui;FAN Jinxin(Jiangxi Provincial Children's Hospital,Nanchang,330006)

机构地区：[1]江西省儿童医院,330006

出　　处：《实用癌症杂志》2025年第3期428-432,共5页The Practical Journal of Cancer

基　　金：江西省卫生健康委科技计划项目(编号:202311079,202311080)。

摘　　要：目的 分析儿童梭形细胞型滑膜肉瘤(SS)的临床病理学特征、诊断及鉴别诊断。方法 回顾性分析3例梭形细胞型滑膜肉瘤的临床病理学特征、免疫表型和分子检测结果。结果 镜下肿瘤与周围组织分界不清,梭形瘤细胞,呈束状、鱼骨样、交织状排列,部分区域细胞稀疏、粘液样变。例1复发标本中梭形细胞间可见多少不等的胶原纤维和坏死,并有较多病理性核分裂像。3例均未见上皮细胞巢。免疫表型:3例弥漫表达SS18-SSX、Vimentin、CD99、TLE1、Bcl-2、EGFR(其中1例为复发时阳性)、INI-1弱阳性、局灶或部分病例表达Cyclin D1、CK7、EMA、CKpan(1/3)、TRKpan(2/3,细胞质弱+)、CD56(1/3),β-Catenin细胞质阳性。3例分子检测结果均为SS18基因断裂阳性,NTRK1/2/3基因断裂阴性。结论 儿童梭形细胞型SS少见,需要和多种儿童梭形细胞肿瘤进行鉴别。EGFR在儿童梭形细胞型SS中高表达,其意义和机制有待进一步研究。Objective To study the clinicopathological features,diagnosis and differential diagnosis of childhood synovial sarcoma.Methods Retrospective analysis of clinicopathological features,immunophenotypes,and molecular detection results of 3 spindle cell type synovial sarcoma.Results Microscopically,the boundary between the tumor and surrounding tissues was unclear,tumor cells were spindle shaped,arranged in bundles,fishbone like or interwoven shapes,some areas had sparse and mucinous changes.In the recurrent specimen of case 1,there were varying amounts of collagen fibers and necrosis between spindle cells,as well as many pathological mitotic phases.No epithelial cell nests were found in all 3 cases.Immunohistochemically,all 3 cases diffusely expressed SS18-SSX,Vimentin,CD99,TLE1,Bcl-2,EGFR(1 case was positive during recurrence)and INI-1(weak),partially or focally expressed CyclinD1,CK7,EMA,CKpan(1/3),TRKpan(2/3,cytoplasm,weak),CD56(1/3),β-Catenin(cytoplasm,weak).All 3 cases were positive for SS18 gene rearrangement and negative for NTRK1/2/3 rearrangement.Conclusion Spindle cell type SS in childhood is rare and needs to be differentiated from other various spindle cell tumors of children.EGFR is highly expressed in spindle cell type SS of children,and its significance and mechanism need further research.

关 键 词：儿童滑膜肉瘤 梭形细胞型 EGFR抗体 SS18基因 荧光原位杂交(FISH) 

分 类 号：R738.5[医药卫生—肿瘤]