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Surgical Oncology Manual pp 127–136Cite as

Desmoid Fibromatosis and Dermatofibrosarcoma Protuberans

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Abstract

Desmoid-type fibromatosis (DF) and dermatofibrosarcoma protuberans (DFSP) are rare mesenchymal neoplasms with intermediate behavior that can be locally aggressive and in the case of DFSP, rarely metastasizing. Both tumors frequently contain specific mutations that may be useful in diagnosis or treatment with targeted therapies. The management of DF has recently changed from an approach of upfront surgical resection to an initial period of observation. Medical therapies including anti-inflammatories, anti-hormones, cytotoxic chemotherapy and targeted agents are gaining increasing utility. Recurrence is common after resection, especially in intra-abdominal or head and neck DF. In contrast, wide local excision is preferred for resectable DFSP. Targeted agents should be considered for borderline resectable or complex recurrent lesions and for metastatic disease. Fibrosarcomatous progression behaves more aggressively than classic DFSP and requires a multidisciplinary approach. Both DF and DFSP are complex diseases that necessitate multidisciplinary discussion to avoid significant morbidity from extensive resection and minimize recurrence.

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Author information

Authors and Affiliations

  1. University of Toronto, Toronto, ON, Canada

    Trevor D. Hamilton M.D., F.R.C.S.C. (Fellow, General Surgical Oncology)

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

    Brendan Dickson MD, MSc, FCAP, FRCPC

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

    Rebecca A. Gladdy MD, PhD, FRCSC, FACS

Authors
  1. Trevor D. Hamilton M.D., F.R.C.S.C.
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  2. Brendan Dickson MD, MSc, FCAP, FRCPC
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  3. Rebecca A. Gladdy MD, PhD, FRCSC, FACS
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Corresponding author

Correspondence to Trevor D. Hamilton M.D., F.R.C.S.C. .

Editor information

Editors and Affiliations

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

    Frances C. Wright

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

    Jaime Escallon

  3. Department of Surgical Oncology, National Cancer Institute, Panama City, Panama

    Moises Cukier

  4. General Surgical Oncology, University of Toronto, 1X5, Ontario, Canada

    Melanie E. Tsang

  5. General Surgical Oncology, University of Toronto, Toronto, Ontario, Canada

    Usmaan Hameed

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© 2016 University of Toronto General Surgery Oncology Program

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Hamilton, T.D., Dickson, B., Gladdy, R.A. (2016). 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/10.1007/978-3-319-26276-5_9

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  • DOI: https://doi.org/10.1007/978-3-319-26276-5_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-26274-1

  • Online ISBN: 978-3-319-26276-5

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