Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy.,Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy. Electronic address: marco.fiore@istitutotumori.mi.it.,Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy; Department of Surgical Oncology, Mount Sinai Hospital and Princess Margaret Cancer Centre, and Department of Surgery, University of Toronto, 600 University Avenue, Toronto, Ontario M5G 1X5, Canada.
Publication
Summary
Surgery has been the historical standard of care but carries considerable drawbacks in terms of high local recurrence rates and poor functional outcomes. Improved understanding of the natural history of desmoid-type fibromatosis has resulted in a paradigm shift toward nonoperative management. Effective medical treatment options include nonsteroidal antiinflammatory drugs, hormone therapy, cytotoxic chemotherapy, and targeted agents. A treatment algorithm has been proposed with the objective of optimizing treatment.
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