儿童炎性肌纤维母细胞瘤11例临床病理分析

目的 探讨儿童炎性肌纤维母细胞瘤(IMT)的临床病理特征和免疫组化表型.方法 分析11例儿童炎性肌纤维母细胞瘤的镜下特征和免疫组化表达,并进行文献复习.结果 本组男性5例,女性6例,主要发生于腹、臀、肾上腺、颈及会阴,临床症状与部位相关.组织学显示梭形纤维母细胞和肌纤维母细胞疏密混杂排列,有黏液样基质及炎性细胞浸润.免疫组化:肿瘤细胞vimentin、SMA(+),desmin、NSE和MSA大部分(+);5例ALK-1(+),2例CK(+);S-100蛋白和CD34(-).结论 儿童IMT是一种常见的良性肿瘤,复发少见,但有恶变潜能.

Inflammatory myofibroblastic tumor in children:a clinicopathologic study of 11 cases

Objective To investigate the clinicopathological characteristics of the inflammatory myofibroblastic tumor(IMT) in children with literalure review.Methods Eleven cases of IMT in children were analysed by light microscopy and immunostaining.Results The age of the five male and six female cases was from 5 months to 10 years,with the mean of 4 years.The sites of IMT included abdomen,buttock,vulvar,adrenal gland and neck.Histologically,the tumor was characterisized by mixed arrangements of spindle cells,fibroblast and myofibroblast,varying degree of myxiod background and infiltration of inflammatory cells.Immunohistochemical staining showed that the tumor cells were positive for vimentin,SMA,desmin,NSE,MSA(2/3 cases+,70%);ALK-1 was positive in 5 cases and CK in 2 cases,but none case expressed S-100 and CD34.Conclusion IMT is a special soft tissue tumors and mostly benign,which most commonly affects children with recurrent potential.