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Fibrohistiocytic Skin Cancers: Dermatofibrosarcoma Protuberans, Atypical Fibroxanthoma, and Undifferentiated Pleomorphic Sarcoma

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Abstract

As a group, atypical fibroxanthoma, undifferentiated pleomorphic sarcoma, and dermatofibrosarcoma protuberans (DFSP) are rare cutaneous neoplasms with the potential for significant local destruction. In DFSP, delayed diagnosis is common as the clinical presentation can appear benign. Tissue stains are key for diagnosis of dermatofibrosarcoma protuberans from the benign histologic mimicker dermatofibroma. Debate exists regarding the relationship between atypical fibroxanthoma and undifferentiated pleomorphic sarcoma. The preferred treatment for fibrohistiocytic tumors is Mohs micrographic surgery. Since close monitoring for recurrence is critical, repair options for these commonly large postsurgical defects must be carefully planned. In all cases, preoperative planning and management of patient expectation is paramount to successful therapy.

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Authors and Affiliations

  1. Mariwalla Dermatology, West Islip, NY, USA

    Kavita Mariwalla

  2. Department of Medicine, Division of Dermatology, Vanderbilt University Medical Center, Nashville, TN, USA

    Allison Hanlon

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  1. Kavita Mariwalla
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Correspondence to Allison Hanlon .

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  1. Department of Medicine, Division of Dermatology, Vanderbilt University Medical Center, Nashville, Tennessee, USA

    Allison Hanlon

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Mariwalla, K., Hanlon, A. (2018). Fibrohistiocytic Skin Cancers: Dermatofibrosarcoma Protuberans, Atypical Fibroxanthoma, and Undifferentiated Pleomorphic Sarcoma. In: Hanlon, A. (eds) A Practical Guide to Skin Cancer. Springer, Cham. https://doi.org/10.1007/978-3-319-74903-7_9

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  • DOI: https://doi.org/10.1007/978-3-319-74903-7_9

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