多中心性婴幼儿肌纤维瘤病  

Multicentric infantile myofibromatosis:a case report

作　　者：姚树兰[1] 杨纯丽 李岩[2] 田中华[1] 卢彬[1] YAO Shulan;YANG Chunli;LI Yan;TIAN Zhonghua;LU Bin(Department of Dermatology,Affiliated Hospital of Jining Medical University,Jining 272029,China;Department of Pathology,Affiliated Hospital of Jining Medical University,Jining 272029,China)

机构地区：[1]济宁医学院附属医院皮肤科,山东济宁272029 [2]济宁医学院附属医院病理科,山东济宁272029

出　　处：《临床皮肤科杂志》2025年第1期34-36,共3页Journal of Clinical Dermatology

摘　　要：报告1例多中心性婴幼儿肌纤维瘤病。患儿男,5个月龄。出生后6 d全身出现散在结节并逐渐增大。皮肤科检查:全身散在数个类圆形红色结节,界限清楚,表面光滑,毛细血管扩张,质硬。外院颅脑CT及胸部X射线检查均未见明显骨质破坏。皮损组织病理检查:表皮大致正常,肿瘤位于真皮中下部,由2类细胞构成,一类为梭形细胞呈结节状及漩涡状排列,胞质嗜酸性,具有细长或泡状核;一类为圆形或小多边形的原始间叶细胞,呈实性片状分布或围绕血管呈血管外皮瘤样,未见坏死和细胞异形性。免疫组化示平滑肌肌动蛋白(SMA)阳性。诊断:多中心性婴幼儿肌纤维瘤病。随访3个月,部分结节缩小。A case of multicentric infantile myofibromatosis is reported.A 5-month-old male infant presented with multiple nodules on the skin,which appeared at six days after birth and gradually enlarged thereafter.Dermatological examination showed several round,red nodules that were scattered across his body surface.The nodules were well-defined and had a smooth sur face with telangiectasia,accompanied by a hard texture.His previous skull computed tomography and chest X-ray results at another hospital showed no obvious bone destruction.Histopathological examination showed that the epidermis was mostly normal,and the tumour was located in the middle and lower dermis.The tumour was composed of two types of cells:spindle cells,which exhibited nodular and vortex-like arrangement,eosinophilic cytoplasm,and elongated or vesicular nuclei;and primitive mesenchymal cells,which had a round or small polygonal shape and were distributed in solid sheets or hemangiopericytoma in the blood vessels,without necrosis or cell atypia.Immunohistochemistry results showed positive smooth muscle actin.A diagnosis of multicentric infantile myofibromatosis was made.Several nodules subsided after three months of follow-up.

关 键 词：肌纤维瘤病 婴幼儿 

分 类 号：R738.7[医药卫生—肿瘤]