title: A systematic review of active treatment options in patients with desmoid tumours.
abstract: BACKGROUND
  We conducted a systematic review to determine the optimal treatment options in patients with desmoid tumours who have declined observational management.
  
  
  METHODS
  A search was conducted of the medline and embase databases (1990 to September 2012), the Cochrane Library, and relevant guideline Web sites and conference materials.
  
  
  RESULTS
  One systematic review and forty-six studies met the preplanned study selection criteria; data from twenty-eight articles were extracted and analyzed. For local control, three studies reported a statistically significant difference in favour of surgery plus radiotherapy (rt) compared with surgery alone, and one study did not; two studies reported the lack of a statistical difference between surgery plus rt and rt alone in maintaining local control. Multivariate risk factors for local recurrence included positive surgical margins and young patient age. Single-agent imatinib led to a progression-free survival rate of 55% at 2 years and 58% at 3 years. Methotrexate plus vinblastine led to a progression-free survival rate of 67% at 10 years. Significant toxicities were reported for all treatment modalities, including surgical morbidity, and rt- and chemotherapy-related toxicities.
  
  
  CONCLUSIONS
  In patients who have declined observational management, the local control rate was higher with surgery plus rt than with surgery alone. However, the additional rt-related complications should be considered in treatment decision-making. Surgery, rt, and systemic therapy are all reasonable treatment options for patients with desmoid tumours.
authors.affiliation: Cancer Care Ontario's Program in Evidence-Based Care, Department of Oncology, McMaster University, Hamilton, ON.
authors.forename: X
authors.initials: X
authors.lastname: Yao
authors.affiliation: Division of Radiation Oncology, Juravinski Cancer Centre, Hamilton, ON.
authors.forename: T
authors.initials: T
authors.lastname: Corbett
authors.affiliation: Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON.
authors.forename: A A
authors.initials: AA
authors.lastname: Gupta
authors.affiliation: Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, ON.
authors.forename: R A
authors.initials: RA
authors.lastname: Kandel
authors.affiliation: Department of Medical Oncology, The Ottawa Hospital Regional Cancer Centre, Ottawa, ON.
authors.forename: S
authors.initials: S
authors.lastname: Verma
authors.affiliation: Department of Orthopaedic Surgery, The Ottawa Hospital Regional Cancer Centre, Ottawa, ON.
authors.forename: J
authors.initials: J
authors.lastname: Werier
authors.affiliation: Division of Orthopaedic Surgery, Juravinski.
authors.forename: M
authors.initials: M
authors.lastname: Ghert
keywords: Desmoid tumours; systematic review; treatments
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