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Desmoid Fibromatosis and Dermatofibrosarcoma Protuberans

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Surgical Oncology Manual

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Abstract

Desmoid tumors (DTs) and dermatofibrosarcoma protuberans (DFSP) are rare mesenchymal neoplasms. Most DTs arise sporadically, but 5–10% are associated with familial adenomatous polyposis (FAP). Management has shifted from upfront surgical resection to active surveillance. If DTs progress during active surveillance, then medical therapies can be initiated based on patient preference and toxicity. Surgical resection is limited to preservation of function and is implemented after multidisciplinary discussion. Recurrent DT is similarly managed initially with active surveillance.

DFSP is the most common dermal sarcoma. Fibrosarcomatous transformation (FS-DFSP) is associated with an increased chance of distant metastases and occurs in 5–15% of DFSP patients. Primary management of DFSP is wide local excision (WLE), ideally with margins of 2–3 cm to minimize local recurrence. Medical management with imatinib can be used in the neoadjuvant setting for locally advanced disease or in the management of metastatic disease.

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Author information

Authors and Affiliations

  1. General Surgical Oncology, University of Toronto, Toronto, ON, Canada

    Ricky Jrearz

  2. Medical Oncology, University of Toronto, Toronto, ON, Canada

    Samir Fasih

  3. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada

    Brendan C. Dickson

  4. Division of Medical Oncology, University of Toronto, Toronto, ON, Canada

    Abha A. Gupta

  5. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Rebecca A. Gladdy

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  1. Ricky Jrearz
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  2. Samir Fasih
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  3. Brendan C. Dickson
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  4. Abha A. Gupta
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  5. Rebecca A. Gladdy
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Correspondence to Rebecca A. Gladdy .

Editor information

Editors and Affiliations

  1. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Frances C. Wright

  2. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Jaime M. Escallon

  3. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Moises Cukier

  4. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Melanie E. Tsang

  5. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Usmaan Hameed

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Jrearz, R., Fasih, S., Dickson, B.C., Gupta, A.A., Gladdy, R.A. (2020). Desmoid Fibromatosis and Dermatofibrosarcoma Protuberans. In: Wright, F., Escallon, J., Cukier, M., Tsang, M., Hameed, U. (eds) Surgical Oncology Manual. Springer, Cham. https://doi.org/10.1007/978-3-030-48363-0_8

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