title: The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network.
abstract: OBJECTIVE
  A multi-centre study to assess the value of combined surgical resection and radiotherapy for the treatment of desmoid tumours.
  
  
  METHODS
  One hundred and ten patients from several European countries qualified for this study. Pathology slides of all patients were reviewed by an independent pathologist. Sixty-eight patients received post-operative radiotherapy and 42 surgery only. Median follow-up was 6 years (1 to 44). The progression-free survival time (PFS) and prognostic factors were analysed.
  
  
  RESULTS
  The combined treatment with radiotherapy showed a significantly longer progression-free survival than surgical resection alone (p smaller than 0.001). Extremities could be preserved in all patients treated with combined surgery and radiotherapy for tumours located in the limb, whereas amputation was necessary for 23% of patients treated with surgery alone. A comparison of PFS for tumour locations proved the abdominal wall to be a positive prognostic factor and a localization in the extremities to be a negative prognostic factor. Additional irradiation, a fraction size larger than or equal to 2 Gy and a total dose larger than 50 Gy to the tumour were found to be positive prognostic factors with a significantly lower risk for a recurrence in the univariate analysis. This analysis revealed radiotherapy at recurrence as a significantly worse prognostic factor compared with adjuvant radiotherapy. The addition of radiotherapy to the treatment concept was a positive prognostic factor in the multivariate analysis.
  
  
  CONCLUSIONS
  Postoperative radiotherapy significantly improved the PFS compared to surgery alone. Therefore it should always be considered after a non-radical tumour resection and should be given preferably in an adjuvant setting. It is effective in limb preservation and for preserving the function of joints in situations where surgery alone would result in deficits, which is especially important in young patients.
authors.affiliation: Radiation Oncology, University Hospital Zurich, Switzerland. brigitta.baumert@maastro.nl
authors.forename: Brigitta G
authors.initials: BG
authors.lastname: Baumert
authors.forename: Martin O
authors.initials: MO
authors.lastname: Spahr
authors.forename: Arthur
authors.initials: A
authors.lastname: Von Hochstetter
authors.forename: Sylvie
authors.initials: S
authors.lastname: Beauvois
authors.forename: Christine
authors.initials: C
authors.lastname: Landmann
authors.forename: Katrin
authors.initials: K
authors.lastname: Fridrich
authors.forename: Salvador
authors.initials: S
authors.lastname: Villà
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Kirschner
authors.forename: Guy
authors.initials: G
authors.lastname: Storme
authors.forename: Peter
authors.initials: P
authors.lastname: Thum
authors.forename: Hans K
authors.initials: HK
authors.lastname: Streuli
authors.forename: Norbert
authors.initials: N
authors.lastname: Lombriser
authors.forename: Robert
authors.initials: R
authors.lastname: Maurer
authors.forename: Gerhard
authors.initials: G
authors.lastname: Ries
authors.forename: Ernst-Arnold
authors.initials: EA
authors.lastname: Bleher
authors.forename: Alfred
authors.initials: A
authors.lastname: Willi
authors.forename: Juerg
authors.initials: J
authors.lastname: Allemann
authors.forename: Ulrich
authors.initials: U
authors.lastname: Buehler
authors.forename: Hugo
authors.initials: H
authors.lastname: Blessing
authors.forename: Urs M
authors.initials: UM
authors.lastname: Luetolf
authors.forename: J Bernard
authors.initials: JB
authors.lastname: Davis
authors.forename: Burkhardt
authors.initials: B
authors.lastname: Seifert
authors.forename: Manfred
authors.initials: M
authors.lastname: Infanger
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005060:Europe; D018222:Desmoid Tumors / Q000532:radiotherapy* / Q000601:surgery*; D006801:Humans; D007223:Infant; D008875:Middle Aged; D011878:Radiotherapy / Q000379:methods*; D012008:Recurrence; D012983:Soft Tissue Neoplasms / Q000532:radiotherapy* / Q000601:surgery*; D013997:Time Factors