中国麻风皮肤病杂志 ›› 2026, Vol. 42 ›› Issue (10): 737-740.doi: 10.12144/zgmfskin202610737

• 病例报告 • 上一篇    下一篇

儿童臀部隆突性皮肤纤维肉瘤一例

邓乐曲,唐枭伟,朱云峰,李坤,龙湫子,李磊   

  1. 南京医科大学第二附属医院烧伤整形科, 江苏南京,210000
  • 出版日期:2026-10-15 发布日期:2026-10-09

Dermatofibrosarcoma protuberans in the buttock of a child: a case report

DENG Yuequ, TANG Xiaowei, ZHU Yunfeng, LI Kun, LONG Qiuzi, LI Lei   

  1. Department of Burn and Plastic Surgery, the Second Affiliated Hospital of Nanjing Medical University, Nanjing 210000, China
  • Online:2026-10-15 Published:2026-10-09

摘要: 隆突性皮肤纤维肉瘤是一种低度恶性梭形细胞间质肿瘤,儿童发病罕见。本文报道1例4岁女性患儿,自1岁起臀部同一部位反复外伤,每次伤后均出现血肿样肿物,经换药对症处理后皮损可逐步消退。1个月前患儿臀部再次外伤后,同部位出现一约4 cm×3 cm×1 cm肿物,呈褐青色,边界清晰,后因肿物破溃继发感染,于外院行肿物切除,术后创面愈合不佳。经多家医院病理会诊,最终确诊为隆突性皮肤纤维肉瘤,分子生物学检测提示COL1A1-exon45-PDGFB-exon1融合,变异率为9.15%。该患儿予肿瘤扩大切除术,术后辅以放疗,随访2年未复发。

关键词: 隆突性皮肤纤维肉瘤, 儿童, 扩大切除

Abstract: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant spindle cell mesenchymal tumor with rare occurrence in children. This study reports a 4-year-old female patient who had recurrent trauma at the same site of the buttock since the age of 1, with repeated hematoma-like masses forming after each injury and healing after dressing changes. One month prior to presentation, a 4 cm×3 cm×1 cm bruise-blue, well-circumscribed hematoma-like mass developed at the identical buttock site following recurrent trauma. The mass ruptured and became infected, with poor wound healing after tumor resection performed at an external hospital. The diagnosis of DFSP was confirmed via pathological consultation and immunohistochemical examination from multiple institutions. Molecular biological testing identified the COL1A1-exon45-PDGFB-exon1 fusion with a variant rate of 9.15%. The patient was treated with wide local excision of the tumor combined with postoperative radiotherapy, and no recurrence was observed during the two-year follow-up period.

Key words: dermatofibrosarcoma protuberans, child, extended resection