婴儿肢端纤维瘤病11例临床分析

Clinical Analysis of 11 Cases of Infantile Digital Fibromatosis

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。

Objective To analyze the clinical features of infantile digital fibromatosis（IDF）, and to investigate how to choose treatment plans. Methods We retrospectively analyzed 11 cases of patients with IDF that were treated at the department of dermatology, Beijing Children＇s Hospital between 2007 and 2016. Results Of the 11cases, 5 cases were male, 6 cases were female. Average age of onset was 2.85 months（0～8months）. All the lesions were characterized by single or multiple, flesh-colored or pink, firm, smooth and dome-shaped nodule measuring 1.26cm averagely, on the extensor or lateral aspect of the digits.Four lesions were symptomatic and treated with surgery. Two lesions were rapidly growing（size enlarged up to more than two times in 4 weeks）, and treated with intra-lesional steroids. Other 5 lesions were asymptomatic and slowly growing relatively,which were followed up until spontaneous regression. Before operation, all the lesions conducted skin biopsies and histopathologic examinations revealed dermal mass of uniform spindle cells and collagen bundles arranged ininterlacing fascicles and the spindle cells with round eosinophilic inclusion body can be seen. All the patients achieved good treatment effects.Conclusion IDF have typical clinical presentations and histopathological features. Our suggestion about choice of treatment plan was that for most of asymptomatic patients, follow-up was as the first-line treatment; for patients, who were symptomatic, surgery should be considered; for patients, whose lesions were rapidly growing or had the risk of dysfunctions, intra-lesional streroids should be taken. We need to further expend sample size and long-term follow-up all the patients.