儿童丛状血管瘤15例临床、影像及病理特征分析  

Clinical,Imaging and Pathological Analysis of 15 Cases of Tufted Angioma in Children

作　　者：周念[1] 夭志刚 浦芸静 张莉[1] 李艳 舒虹[1] ZHOU Nian;YAO Zhigang;PU Yunjing;ZHANG Li;LI Yan;SHU Hong(Department of Dermatology,Kunming Children's Hospital,Kunming 650034,China;Department of Urology Surgery,Kunming Children's Hospital,Kunming 650034,China)

机构地区：[1]昆明市儿童医院皮肤科,云南昆明650034 [2]昆明市儿童医院泌尿外科,云南昆明650034

出　　处：《中国皮肤性病学杂志》2024年第5期516-521,530,共7页The Chinese Journal of Dermatovenereology

摘　　要：目的 提高临床医生对儿童丛状血管瘤的认识。方法 回顾性分析昆明市儿童医院皮肤科2018年1月—2023年1月收治的15例丛状血管瘤患儿的临床、影像及病理资料特征。结果 15例丛状血管瘤患儿中,男11例(73.33%),女4例(26.67%),患儿发病年龄为0~5岁8个月。12例在1岁内患病,3例1岁以后患病。皮损位于头颈部6例、躯干、四肢9例。皮损首发时常以界限不清的暗红或紫红的浸润性斑片、斑块、结节为主,质地韧或硬,面积逐渐扩大,2例伴有局部多毛,5例皮损触痛,2例合并卡梅现象(Kasabach-Merritt phenomenon,KMP),1例伴有受累肢体活动障碍。皮肤镜下2例可见毳毛及黄白色角栓样结构,10例可见不典型浅棕色网状色素结构,15例背景均为淡红色背景,部分可见少许线状、分支状及点状血管影。超声检查12例可见丰富的条棒状血流,2例未见血流信号,1例可见星点状血流。10例患儿行磁共振成像(MRI)均表现为T2WI高信号,增强扫描明显强化。组织病理特点均为真皮内分布由血管内皮细胞和外皮细胞组成的多个结节或小叶,呈同心漩涡状或“炮弹”样生长,细胞无异型性。结论 丛状血管瘤临床较为罕见,诊断主要靠组织病理检查,超声、皮肤镜可为临床诊断提供线索,MRI可评估受累范围,因部分患者可伴有KMP,因此需严密随访。Objective To better characterize the clinical findings of tufted angioma(TA) in children.Methods The clinical presentation,image and histopathological findings of 15 patients with TA in Dermatology Department of Kunming Children′s Hospital from January 2018 to January 2023 were analysed retrospectively.Results Among the 15 children with TA,there were 11 males(73.33%) and 4 females(26.67%).The age ranged from 0 month to 5 years and 8 months old.The attack time of 12 cases with TA within the age of 1 year,and 3 cases after the age of 1 year.The lesions of 6 cases distributed on the head and neck,and 9 cases on the trunk and extremities.The initial appearance of skin lesions often consists of indistinct dark red or purplish red infiltrating patches,plaques,and nodules,with tough or hard texture and gradually expanding.Two cases were accompanied by hirsutism,five cases were tenderness,two cases were accompanied by Kasabach-Merritt phenomenon(KMP),and one case was accompanied by affected limb movement disorders.Under dermatoscopy,two cases showed vellus hair and yellow white horn like structures,10 cases showed atypical light brown reticular pigment structures,and 15 cases had a light red background,with slight linear,branching,and punctate vascular shadows.Ultrasound examination showed abundant rod-shaped blood flow in 12 cases,no blood flow signal in two cases,and punctiform blood flow in 1 case.All 10 children showed high T2WI signal on MRI,with significant enhancement on enhanced scans.The pathological characteristics of the tissue were multiple nodules or lobules composed of vascular endothelial cells and outer skin cells distributed within the dermis,growing in a concentric vortex like or "shell like" shape,with no cell atypia.Conclusion TA is a relatively rare disease,and its diagnosis mainly based on histopathology.Ultrasound and dermatoscopy can provide clues for diagnosis,and MRI can evaluate the affected area.As some patients may have KMP,follow-up is necessary.

关 键 词：丛状血管瘤 临床表现 影像学特征 组织病理 回顾性分析 

分 类 号：R751[医药卫生—皮肤病学与性病学] R739.5[医药卫生—临床医学]