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Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous neoplasm with intermediate- to low-grade malignancy, but can be a surgical challenge. DFSP is characterized by a storiform proliferation of bland-appearing cells that diffusely infiltrate the dermis. Due to these characteristics, it has a high potential for wide and deep extensions into the deep dermis. Although the majority of DFSPs are cured by Mohs micrographic surgery, tumor size and disfigurement may be a limiting factor for such a procedure. Moreover, even when surgery is utilized, there are cases where positive margins remain, in which case imatinib would be an option, often in combination with surgery. Imatinib can decrease tumor size preoperatively and help to improve postsurgical aesthetic appearance and minimize functional impairment. This article is a review of literature examining the indications for the use of imatinib in DFSP lesions and understanding the pathophysiology behind the treatment.
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