乳房に生じた線維肉腫成分を伴う隆起性皮膚線維肉腫の1例

Fibrosarcomatous Dermatofibrosarcoma Protuberans in the Breast

はじめに

　隆起性皮膚線維肉腫（dermatofibrosarcoma protuberans：以下，DFSP）は真皮から皮下組織に由来する比較的まれな軟部腫瘍で，20～40歳代の成人に好発し，紅斑や隆起性結節を形成する病変である 1）。DFSPの亜型としてfibrosarcomatous DFSP（以下，DFSP-FS）があり，DFSPの約10 ％を占める。DFSP-FSは通常のDFSPよりも局所再発率が高く，高率に遠隔転移を来たし 2），その予後は不良とされる 3）。今回われわれは，乳房に生じたDFSP-FSを経験したため，文献的考察を加えて報告する。

　Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor originating from the dermis to subcutaneous tissue. Compared to classical DFSP, fibrosarcomatous DFSP (DFSP-FS) has a higher local recurrence rate, a higher incidence of distant metastasis, and a poorer prognosis. We report a case of DFSP-FS in the breast of a 21-year-old female with no prior medical history. The diagnosis of DFSP-FS was confirmed through a partial biopsy, which included immunohistochemical staining and FISH analysis. A wide incisional excision with a 2 cm margin was performed, and the resulting skin defect was treated with a split-thickness skin graft.