儿童皮肤硬化性纤维瘤病  

A case report of skin desmoid fibromatosis in childhood

作　　者：余音[1] 王苏平[1] 肖沙[1] 郝进[1] 

机构地区：[1]重庆市中医院皮肤科,重庆400011

出　　处：《临床皮肤科杂志》2015年第3期158-160,共3页Journal of Clinical Dermatology

摘　　要：报告1例少见的儿童皮肤硬化性纤维瘤病。患儿女,5岁。因右大腿斑块1年余就诊。家族中无同病患者。体格检查:右大腿根部见一直径约1.5 cm的环形红斑,表面光滑,质硬。皮损组织病理示真皮内大量密集排列的梭形细胞增生,未见明显核分裂象及坏死,缺乏假包膜呈局部浸润性生长,伴不同程度胶原化。免疫组化检查:游离人β-连环蛋白(β-catenin)(+),波形蛋白(vimetin)(+),CD34灶状(+),S-100蛋白、平滑肌肌动蛋白(SMA)、上皮膜抗原(EMA)、CD10、髓鞘碱性蛋白(MBP)、神经烯醇化酶(NSE)阴性。。诊断:皮肤硬化性纤维瘤病。A rare case of skin desmoid fibromatosis in childhood was reported. A 5-year-old child presented with a tumor on the root of her right thigh for more than 1 year without any pain or itch. Nobody else in her family had the same dis- ease. Physical examinations showed the patient was otherwise healthy and a diameter of 1.5cm in size,annular,solitary,hard, red-colored tumor could be seen on the root of her right thigh. Histological examinations showed a large number of spindle cells densely proliferated and packed in the dermis without obvious mitotic phase and necrosis,which locally invasivly grew with different levels of collagenization and a lack of pseudocapsule. The immunohistochemistry showed β-catenin（＋）,Vim（＋）, CD34（＋）in focaI,S-100（-）,SMA（-）,EMA（-）,CDI0（-）,MBP（-）,NSE（-）. These features were consistent with desmoid fibro- matosis.

关 键 词：硬化性纤维瘤病 皮肤 儿童 

分 类 号：R739.5[医药卫生—肿瘤]