婴儿肢端纤维瘤病11例临床分析

发布时间：2018-02-21 02:59

本文关键词： 婴儿肢端纤维瘤病包涵体　出处：《中国皮肤性病学杂志》2017年07期 　论文类型：期刊论文

【摘要】：目的了解婴儿肢端纤维瘤病临床特点,探讨治疗方案选择依据。方法回顾性分析北京儿童医院皮肤科2007-2016年诊治的11例婴儿肢端纤维瘤病患儿的临床资料。结果 11例患儿,男5例,女6例。平均发病年龄2.85月(0~8个月)。均表现为坚实、平滑、圆顶状、肤色或淡粉色的结节,位于指趾背面或侧面。肿瘤直径平均1.26cm(0.6~2.8cm)。4例出现临床症状,均予手术治疗。2例皮损增长迅速(4周内增长2倍以上),予瘤体内注射得宝松治疗。余5例无任何症状且生长相对较慢,密切随诊。所有患儿术前均行活检,病理示瘤体由增生的梭形细胞构成,呈束状或编织样排列,其间夹有胶原纤维,肿瘤细胞胞质内可见红色圆形嗜伊红包涵体。患儿均取得良好疗效。结论婴儿肢端纤维瘤病临床及组织病理特点典型,多数无症状患儿可密切随访,有症状者手术治疗可取得良好效果,生长迅速的皮损予糖皮质激素注射治疗也可获得满意疗效。但仍需扩大样本量论证及长期随访。
[Abstract]:Objective to understand the clinical characteristics of infantile limb fibromatosis and to explore the basis of treatment. Methods the clinical data of 11 cases of infantile extremity fibromatosis treated by dermatology department of Beijing Children's Hospital from 2007 to 2016 were retrospectively analyzed. Results 11 cases of infantile extremity fibromatosis were treated and treated in Beijing Children's Hospital from 2007 to 2016. There were 5 males and 6 females. The mean age of onset was 2. 85 months or 8 months. All of them showed solid, smooth, domed nodule, skin color or light pink nodule. The mean diameter of the tumor was 1. 26 cm, 0. 6, 2. 8 cm and 4. 4 cases appeared clinical symptoms, and the mean diameter of the tumor was 1. 26 cm and 0. 6% or 2. 8 cm. All the cases were treated with surgical treatment. The skin lesions increased more than 2 times in 4 weeks, and were treated by intratumoral injection of diprasone. The remaining 5 cases had no symptoms and grew relatively slowly. All the children were given biopsy before operation. Pathology showed that the tumor was composed of proliferating fusiform cells, arranged in bundles or braided, with collagenous fibers in them. Red round eosinophilic inclusion bodies can be seen in the cytoplasm of tumor cells. Conclusion the clinical and histopathological characteristics of infantile limb fibromatosis are typical, and most asymptomatic children can be closely followed up. The surgical treatment of symptomatic patients can obtain good results, and the treatment of rapidly growing skin lesions with glucocorticoid injection can also obtain satisfactory results, but it is still necessary to expand the sample size and long-term follow-up.
【作者单位】：首都医科大学附属北京儿童医院皮肤科;
【基金】：北京市医院管理局临床医学发展专项经费资助(ZYLX201601)
【分类号】：R739.5

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