本文關(guān)鍵詞： 嬰兒 肢端 纖維瘤病 包涵體　出處：《中國皮膚性病學(xué)雜志》2017年07期 　論文類型：期刊論文

【摘要】：目的了解嬰兒肢端纖維瘤病臨床特點(diǎn),探討治療方案選擇依據(jù)。方法回顧性分析北京兒童醫(yī)院皮膚科2007-2016年診治的11例嬰兒肢端纖維瘤病患兒的臨床資料。結(jié)果 11例患兒,男5例,女6例。平均發(fā)病年齡2.85月(0~8個月)。均表現(xiàn)為堅(jiān)實(shí)、平滑、圓頂狀、膚色或淡粉色的結(jié)節(jié),位于指趾背面或側(cè)面。腫瘤直徑平均1.26cm(0.6~2.8cm)。4例出現(xiàn)臨床癥狀,均予手術(shù)治療。2例皮損增長迅速(4周內(nèi)增長2倍以上),予瘤體內(nèi)注射得寶松治療。余5例無任何癥狀且生長相對較慢,密切隨診。所有患兒術(shù)前均行活檢,病理示瘤體由增生的梭形細(xì)胞構(gòu)成,呈束狀或編織樣排列,其間夾有膠原纖維,腫瘤細(xì)胞胞質(zhì)內(nèi)可見紅色圓形嗜伊紅包涵體�；純壕〉昧己茂熜�。結(jié)論嬰兒肢端纖維瘤病臨床及組織病理特點(diǎn)典型,多數(shù)無癥狀患兒可密切隨訪,有癥狀者手術(shù)治療可取得良好效果,生長迅速的皮損予糖皮質(zhì)激素注射治療也可獲得滿意療效。但仍需擴(kuò)大樣本量論證及長期隨訪。
[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.
【作者單位】： 首都醫(yī)科大學(xué)附屬北京兒童醫(yī)院皮膚科;
【基金】：北京市醫(yī)院管理局臨床醫(yī)學(xué)發(fā)展專項(xiàng)經(jīng)費(fèi)資助(ZYLX201601)
【分類號】：R739.5

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