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Dermatofibrosarcoma Protuberans

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Mohs Micrographic Surgery

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Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare but locally aggressive spindle cell neoplasm accounting for less than 2% of all soft tissue sarcomas and less than 0.1% of all malignancies. Immunohistochemical and ultrastructural studies indicate that the tumor is of a fibroblastic origin. DFSP commonly occurs in the dermis and has the potential to invade through fascial planes into muscle and bone. It may occur on any part of the body with a predilection for the trunk. The tumor rarely exhibits metastasis, but it has the propensity for subclinical involvement. Complete surgical excision is the mainstay of therapy. DFSP can be resected with either wide local excision (WLE) or Mohs micrographic surgery (MMS). The exact role of neoadjuvant imatinib mesylate in the management of DFSP remains unclear. Successful treatment outcome is dependent on achieving negative surgical margins while preventing functional deficits.

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Authors and Affiliations

  1. Department of Dermatology, Roswell Park Cancer Institute, Buffalo, NY, 14263, USA

    Novie Sroa & Nathalie C. Zeitouni

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  1. Novie Sroa
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  2. Nathalie C. Zeitouni
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Correspondence to Nathalie C. Zeitouni .

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  1. Miller School of Medicine, Dept. Dermatology, University of Miami, NW. 10th Ave. 1600, Miami, 33136, Florida, USA

    Keyvan Nouri

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Sroa, N., Zeitouni, N.C. (2012). Dermatofibrosarcoma Protuberans. In: Nouri, K. (eds) Mohs Micrographic Surgery. Springer, London. https://doi.org/10.1007/978-1-4471-2152-7_19

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  • DOI: https://doi.org/10.1007/978-1-4471-2152-7_19

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