Congenital Dermatofibrosarcoma Protuberans-A Case Report and Literature Review

先天性隆突真皮纖維肉瘤－病例報告及文獻回顧

江原正(Yuan-Cheng Chiang) ； 吳政君(Cheng-Chun Wu) ； 蔡岳儒(Yueh-Ju Tsai) ； 藍叡(Jui Lan)

《臺灣整形外科醫學會雜誌》 23卷1期 (2014/03) Pp. 61-67

摘要

背景：隆突真皮纖維肉瘤是一種少見而低惡性度的腫瘤。主要見於二十至五十歲成年人，兒童非常少見，尤其是先天病例，由於其臨床表現變化大，可能造成延遲診斷。目的及目標：報告一例不尋常之先天性隆突真皮纖維肉瘤病例，並作文獻回顧。材料及方法：一位七歲女童到院時，在其右上胸骨旁有一個四公分大之紅色隆突腫塊。回溯病史時，發現病人出生時，即在胸部上方有一紅色豆子大小之腫塊，此腫塊一直被認為是血管瘤；直到病理檢查證實是隆突真皮纖維肉瘤，遂施與大範圍切除及前外側大皮瓣重建。結果：術後過程良好，二十個月追蹤時無復發情形。結論：先天性隆突真皮纖維肉瘤病例非常少見，由於其臨床表現變化大，或可能呈現類似血管瘤之外觀，造成延遲診斷並不罕見。大範圍切除至病理檢查證實邊緣乾淨，或行莫氏顯微圖手術Mohs micrographic surgery是合適之治療方式。

並列摘要

Background:Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant tumor, which presents mainly in adults at the age of 20 to 50. It is extremely rare in children, especially in congenital cases. Due to its variable clinical presentation, delayed diagnosis is possible.Aim and Objectives:We present a rare, congenital DFSP case and review the literature.Materials and Methods:A 7-year-old girl presented with a 4 × 4 cm, red, prominent mass at right upper parasternal region. Reviewing history, a red, pea-sized small nodule was noted on her upper chest at birth. It was considered a hemangioma thereafter, until pathology proved it to be DFSP. Wide excision and reconstruction with a free anterolateral thigh flap was performed.Results:The postoperative course was smooth. No local recurrence was found at 20 months follow-up.Conclusion:Congenital DFSP is extremely rare. Delayed diagnosis is not uncommon due to its variable clinical presentations including hemangioma-like appearance. Wide local excision with pathologically proven free margin or Mohs micrographic surgery is suggested the optimal treatment.

並列關鍵字

dermatofibrosarcoma protuberans ； congenital