title: Desmoid-Type Fibromatosis: Evolving Treatment Standards.
abstract: Desmoid-type fibromatosis is a rare nonmetastasizing neoplasm with variable behavior. Recent discoveries into the biology of this disease hold promise for identifying prognostic and predictive features and novel therapeutic targets. 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.
authors: Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy. Electronic address: marco.fiore@istitutotumori.mi.it.
authors: Marco
authors: M
authors: Fiore
authors: 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.
authors: Andrea
authors: A
authors: MacNeill
authors: Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy.
authors: Alessandro
authors: A
authors: Gronchi
authors: Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy.
authors: Chiara
authors: C
authors: Colombo
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D019468:Disease Management; D018450:Disease Progression; D018222:Desmoid Tumors / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents
keywords: Aggressive fibromatosis; Chemotherapy; Desmoid tumor; Desmoid-type fibromatosis; Observation; Radiotherapy; Surgery; Wait and see