多中心性婴幼儿肌纤维瘤病
Multicentric infantile myofibromatosis:a case report
姚树兰 1杨纯丽 1李岩 2田中华 1卢彬1
作者信息
- 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
- 折叠
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.
Abstract
A case of multicentric infantile myofibromatosis is reported.A 5-month-old male infant presented with multiple nod-ules 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 nor-mal,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 diagno-sis 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Key words
myofibromatosis/infant引用本文复制引用
出版年
2025
NETL
NSTL
万方数据