Dermatofibrosarcoma Protuberans: Report of a Case

隆突性皮膚纖維肉瘤：病例報告

李妍蒨(Yen-Chien Lee) ； 莊仁賓(Jen-Pin Chuang) ； 林家成(Chia-Cheng Lin) ； 李艷林(Yen-Ling Lee) ； 蘇五洲(Wu-Chou Su) ； 李伯璋(Po-Chang Lee)

《Formosan Journal of Surgery》 40卷5期 (2007/10) Pp. 207-211

https://doi.org/10.29833/FJS.200710.0005

摘要

隆突性皮膚纖維肉瘤是一種生長緩慢且罕見的腫瘤，它與第十七對和第二十二對的染色體重新交換有關。目前治療方式以手術為主，且要留下安全距離，由於此腫瘤對放射線治療有反應，故若無法手術切除乾淨或者安全邊緣過近時，可加上放射線治療。針對腫瘤轉移的病人，可考慮用Imatinib治療。本篇報告一位69歲男性患者，在三年前胸前即有腫塊，最近半年以來，腫塊變大且質地轉硬。手術切除後，病理報告證明為隆突性皮膚纖維肉瘤，安全邊緣僅0.2公分。因為術前未預期為惡性腫瘤，故最靠近安全邊緣位置並未標示。在此種狀態下放射線治療有效與否未有定論，而放射線治療主要的副作用是在心臟血管方面，因為病人的年紀大，且腫瘤的位置正好在胸前，故並未考慮放射線治療。整形外科建議再手術切除乾淨，但病人拒絕，病人至目前為止，已追蹤八個月，沒有復發跡象。此疾病平均復發率為38個月，故尚在宜密切追蹤。

並列摘要

Dermatofibrosarcoma protuberans (DFSP), a rare indolent cutaneous tumor, arises from the rearrangement of chromosomes 17 and 22. The optimal treatment for DFSP is resection with wide margins. DFSP is radiosensitive. Adjuvant radiotherapy may be considered in case of a positive resection margin or close margin. We report a 69-year-old Taiwanese man with DFSP. The lesion began to grow in the front part of the chest wall about 3 years previously. In the past 6 months, the lesion became larger and harder. Surgical resection was performed and the pathology report showed dermatofibrosarcoma with a close margin (about 0.2 cm). For fear of cardiovascular complications, and due to the old age of the patient and the location of the tumor in the front part of the chest wall, radiation therapy was not performed. Eight months after the operation, the patient remained free from recurrence. He is still under close follow up, as the median time of recurrence is around 38 months after surgery.

並列關鍵字

dermatofibrosarcoma protuberans