顏面部隆突性皮膚纖維肉瘤－病例報告

Dermatofibrosarcoma Protuberans of the Face-Case Report and Literature Review

王奕凱(I-Kai Wang) ； 楊安航(An-Han Yan) ； 雷文天(Men-Tin Lui) ； 吳政憲(Cheng-Hsien Wu) ； 高壽延(Shou-Yen Kao)

《中華民國口腔顎面外科學會雜誌》 17卷2期 (2006/09) Pp. 117-127

https://doi.org/10.7104/CJOMS.200609.0117

摘要

隆突性皮膚纖維肉瘤(dermatofibrosarcoma protuberans，DFSP)是一種罕見的低度惡性皮膚腫瘤，它的生長速度雖慢但具有局部侵襲性及容易復發等特性。此種腫瘤好發於軀幹及近端四肢，在頭頸那區域較為少見，所佔比例約12%到15%。在組識學上，腫瘤主要由梭狀細胞嵌於膠原纖維基質，並且排列成特殊的旋渦狀，扇狀或輪狀結構。早期的DFSP常被病人忽視且被醫師視為良性病灶，而保守性的外科手術切除導效相當高比率的局部復發。本篇文章將提出一例台北榮總口腔顎面外科患者其顏面部隆突性皮膚纖維肉瘤之病例報告，對其臨床及病理上的表現、診斷、治療作一敘述並回顧相關文獻。

關鍵字

隆突性皮膚纖維肉瘤 ； 顏面部

並列摘要

Dermatofibrosarcoma protuberans (DPSP) is a rare, slow-growing, locally invasive, low grade mesenchymal malignant neoplasm that originates from the dermis. It usually present as nodular cutaneous mass on the trunk and proximal extremities. Only 12% to 15% of the cases are localized in the head and neck region. Microscopically, it consists of uniform hut atypical spindle cells in a fibrous stroma, forming a characteristic storiform pattern. DFSP is often dismissed by patients and misdiagnosed by physicians as benign lesion in the early stage. Conservative surgical management results in a high local recurrence rate. This paper presents a case of DFSP of the face which was diagnosed and treated at the oral and maxillofacial surgery department of Taipei Veterans General Hospital. A literature review is conducted to highlight the clinicopathological presentation and management of this rare tumor.

並列關鍵字

Dermatofibrosarcoma protuberans DFSP ； Face