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Duan Mengying, Wang Yan, Fang Fang Department of Dermatologic Surgery, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIs, Nanjing 210042, China Corresponding authors: Fang Fang, Email: fangfangjh@126.com; Wang Yan, Email: drwangyan@163.com ��Abstract�� Dermatofibrosarcoma protuberans ��DFSP�� is a rare cutaneous and subcutaneous mesenchymal malignant tumor characterized by high rates of local recurrence and low risk of metastasis. Histopathological and immumohistochemical study can be used to diagnose DFSP. Recent studies have shown that cell genetics and molecular biology, imagiology, dermatoscopy and cutaneous computed tomography ��reflectance confocal microscopy�� can also play an important auxiliary role in the diagnosis, treatment and evaluation of DFSP. At present, the surgical treatment of DFSP mainly includes wide local excision ��WLE�� and Mohs micrographic surgery ��MMS��. Each of the two approaches has its advantages and disadvantages. After surgery, radiation therapy and comprehensive immunological treatment can serve as adjuvant treatment. Cryotherapy is effective in patients with recurrent DFSP, and the targeted agent imatinib can be combined for inoperable patients or patients with positive surgical margins. In addition, antiestrogenic drugs ��such as tamoxifen�� or the new targeted agent heparanase inhibitor roneparstat ��SST0001�� may have some therapeutic effects.

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