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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 understand the clinical features of infant acromegaly and to explore the basis of treatment options. Methods The clinical data of 11 children with acral fibromatosis from Beijing Children’s Hospital Dermatology from 2007 to 2016 were analyzed retrospectively. Results 11 cases of children, 5 males and 6 females. The average age of onset was 2.85 months (0 ~ 8 months). All showed solid, smooth, dome-shaped, complexion or pale pink nodules located on the back or side of the digits. Tumor diameter average 1.26cm (0.6 ~ 2.8cm). 4 cases of clinical symptoms, were treated surgically. 2 cases of rapid increase in skin lesions (4 weeks to increase more than 2 times), to the tumor injected Trepomil treatment. The remaining 5 cases without any symptoms and relatively slow growth, followed up closely. All children underwent biopsy preoperatively. The pathology showed that the tumor consisted of hyperplastic spindle cells arranged in a bundle or braid arrangement with collagen fibers in between. The red circular eosinophilic inclusion bodies were seen in the cytoplasm of tumor cells. Children have achieved good results. Conclusions The clinical and histopathological characteristics of infant acromegaly are typical. Most children with asymptomatic symptoms can follow up closely. Surgical treatment of symptomatic patients can achieve good results. Rapid growth of the lesions can be treated with glucocorticoid injection and satisfactory results can be obtained. But still need to expand the demonstration of sample size and long-term follow-up.