title: Results of a phase II pilot study of moderate dose radiotherapy for inoperable desmoid-type fibromatosis--an EORTC STBSG and ROG study (EORTC 62991-22998).
abstract: BACKGROUND
  To determine the activity of radiotherapy in patients with inoperable desmoid-type fibromatosis (DF) a multicenter prospective phase II trial was carried out.
  
  
  METHODS
  Patients with inoperable progressive disease of primary, recurrent or incompletely resected lesions received a dose of 56 Gy in 28 fractions. Follow-up MRI studies were carried out every 3 months for 2 years and thereafter every 6 months. The primary end point was local control rate at 3 years, estimated by a nonparametric method for interval-censored survival data. Secondary end points were objective tumor response, acute and late toxic effect.
  
  
  RESULTS
  Forty-four patients (27 F/17 M) were enrolled from 2001 to 2008. Median age was 39.5 years. Main tumor sites included trunk 15 (34.1%) and extremities 27 (61.3%). Median follow-up was 4.8 years. The 3-year local control rate was 81.5% (90% one-sided confidence interval 74% to 100%). Best overall response during the first 3 years was complete response (CR) 6 (13.6%), partial response (PR) 16 (36.4%), stable disease 18 (40.9%), progressive disease 3 (6.8%) and nonassessable 1 (2.3%). Five patients developed new lesions. After 3 years, the response further improved in three patients: (CR 2, PR 1). Acute grade 3 side-effects were limited to skin, mucosal membranes and pain. Late toxic effect consisted of mild edema in 10 patients.
  
  
  CONCLUSIONS
  Moderate dose radiotherapy is an effective treatment of patients with DF. Response after radiation therapy is slow with continuing regression seen even after 3 years.
authors.affiliation: Arnhem Radiotherapy Institute, Arnhem. Electronic address: r.keus@arnhemrti.nl.
authors.forename: R B
authors.initials: RB
authors.lastname: Keus
authors.affiliation: Department of Clinical Oncology, LUMC, Leiden, The Netherlands.
authors.forename: R A
authors.initials: RA
authors.lastname: Nout
authors.affiliation: Department of Medicine, Centre Léon Bérard, Lyon, France.
authors.forename: J-Y
authors.initials: JY
authors.lastname: Blay
authors.affiliation: MAASTRO, Maastricht, The Netherlands.
authors.forename: J M
authors.initials: JM
authors.lastname: de Jong
authors.affiliation: Nottingham University Hospitals NHS Trust- City Hospital Campus, Nottingham.
authors.forename: I
authors.initials: I
authors.lastname: Hennig
authors.affiliation: The Royal Marsden NHS Foundation Trust, Surrey, UK.
authors.forename: F
authors.initials: F
authors.lastname: Saran
authors.affiliation: Department of Medicine, Universitaetsklinikum Schleswig-Holstein, University of Kiel, Kiel, Germany.
authors.forename: J T
authors.initials: JT
authors.lastname: Hartmann
authors.affiliation: Department of Neuro-Oncology, Centre Léon Bérard, Lyon, France.
authors.forename: M P
authors.initials: MP
authors.lastname: Sunyach
authors.affiliation: East Surrey Hospital, Surrey and Sussex NHS, Redhill, UK.
authors.forename: S J
authors.initials: SJ
authors.lastname: Gwyther
authors.affiliation: EORTC Headquarters, Brussels, Belgium.
authors.forename: M
authors.initials: M
authors.lastname: Ouali
authors.affiliation: EORTC Headquarters, Brussels, Belgium.
authors.forename: A
authors.initials: A
authors.lastname: Kirkpatrick
authors.affiliation: Department of Radiation Oncology, Institute Verbeeten, Tilburg.
authors.forename: P M
authors.initials: PM
authors.lastname: Poortmans
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden.
authors.forename: P C W
authors.initials: PCW
authors.lastname: Hogendoorn
authors.affiliation: Department of Medical Oncology, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: W T A
authors.initials: WTA
authors.lastname: van der Graaf
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D018222:Desmoid Tumors / Q000000981:diagnostic imaging / Q000532:radiotherapy*; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000532:radiotherapy*; D010865:Pilot Projects; D011446:Prospective Studies; D011859:Radiography; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
keywords: aggressive fibromatosis; desmoid tumors; desmoid-type fibromatosis; radiotherapy; soft tissue tumors