移轉至頸部之纖維肉瘤變異型隆突性皮膚纖維肉瘤

Fibrosarcomatous Variant of Dermatofibrosarcoma Protuberans in Lower Neck

林鴻穎(Horng-Ying Lin) ； 呂宜興(Yi-Shing Leu) ； 陳碧芳(Be-Fong Chen) ； 李國森(Kuo-Sheng Lee)

《台灣耳鼻喉頭頸外科雜誌》 41卷5期 (2006/10) Pp. 177-181

https://doi.org/10.6286/2006.41.5.177

摘要

隆突性皮膚纖維肉瘤是一種罕見的中低惡性度皮膚腫瘤，好局部侵犯且容易局部復發，但鮮少遠端轉移，好發於軀幹及近端四肢，頭頸部較少發生。而纖維肉瘤變異型約佔其10%，臨床上具較高之局部復發率、遠處轉移率與致死率，正確診斷有賴臨床醫師與病理醫師的配合。治療上則以廣泛性切除手術或Mohs顯微手術為主。本病例報告為一名54歲女性，於16年前因右下頸部隆突性皮膚纖維肉瘤於本院接受廣泛腫瘤切除及皮瓣移植術，3年前病人因局部復發接受切片檢查，疑似纖維肉瘤病變，病人無接受進一步治療直至2005年10月，病人因病灶處惡化至本院就醫，經理學及影像檢查發現一10×14cm多發結節合併部分潰瘍，分布於右側下頸部至上胸部，無發現遠端轉移，局部切片為纖維肉瘤，故實施廣泛腫瘤切除及薄皮植皮術，病理報告為纖維肉瘤變異型隆突性皮膚纖維瘤，門診追蹤至今無局部復發現象，特提出報告。

關鍵字

隆突性皮膚纖維肉瘤 ； 纖維肉瘤變異型 ； 頸部

並列摘要

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor of low to intermediate malignant potential, characterized by locally aggressive growth and a high local recurrence rate. However, distant metastases are very rare. This tumor usually develops on the trunk or proximal extremities of patients, but rarely on the head and neck regions. The fibrosarcomatous variant of dermatofibrosarcoma protuberans (FS-DFSP) accounts for approximately 10% of DFSP and is characterized clinically by a higher rate of local recurrence, distant metastasis and tumor related death. The appropriate diagnosis is based on the concurrence of clinicians and pathologists. Wide excision or Mohs micrographic surgery is recommended for the treatment. This report presents a 54-year-old female who received wide excision and flap reconstruction 16 years ago for DFSP on right lower neck. 3 years ago, she got an excisional biopsy of the local recurrent lesion and the histologic examination revealed fibrosacomatous change. She did not accept further treatment until October, 2005. During that time, rapid progression of the recurrent lesion had developed. Physical examination revealed a 10x14 cm area extending from right lower neck to upper chest, which was composed of firm, irregular nodules with some ulcers. There was no distant metastasis from image studies. Histologic finding from biopsy revealed fibrosarcoma. The patient underwent wide excision with split thickness skin grafts, and the pathology report was FS-DFSP. Patient has been free of tumor recurrence till now.

並列關鍵字

dermatofibrosarcoma protuberans ； fibrosarcoma variant ； neck