title: Successful treatment of high risk and recurrent pediatric desmoids using radiation as a component of multimodality therapy.
abstract: OBJECTIVE
  To evaluate the role of radiation therapy (RT) as a component of multimodality therapy for pediatric desmoids.
  
  
  METHODS
  Twenty-one children diagnosed between 1987 and 2005 were identified. Median age at start of treatment was 13 years (range, 2-21). Primary therapy consisted of resection alone (10), resection + external beam radiation therapy (EBRT) (5), resection + chemotherapy (CT; 3), EBRT alone (1), and CT alone (2).
  
  
  RESULTS
  The median follow-up from start of treatment is 75.7 months (range, 16-162). Examining patients with gross total resections (GTRs) (-) margins and those who had GTRs (+) margins followed by EBRT, only 2 of 7 failed primary treatment. Conversely, 13 of 14 patients with other primary treatments failed locally. Of the 15 patients who recurred, only 1 patient had a GTR (-) margins. Seven of these patients had salvage therapy that did not include RT, and of these only 2 have no evidence of disease (NED) at last follow-up. In contrast, the remaining 8 patients received RT as a component of their final salvage therapy and 7 of these are NED at last follow-up. At last follow-up, no patient has died, although toxicities of therapy have occurred.
  
  
  CONCLUSIONS
  Local control is difficult to achieve in pediatric patients with desmoids. In the setting in which negative surgical margins cannot be achieved, RT plays a key role in achieving NED status. Even after multiple recurrences, successful salvage is achievable, particularly when high-dose focal therapy is incorporated.
authors.affiliation: Department of Radiation Oncology, University of California, San Francisco, CA, USA.
authors.forename: Siavash
authors.initials: S
authors.lastname: Jabbari
authors.forename: David
authors.initials: D
authors.lastname: Andolino
authors.forename: Vivian
authors.initials: V
authors.lastname: Weinberg
authors.forename: Brian T
authors.initials: BT
authors.lastname: Missett
authors.forename: Jason
authors.initials: J
authors.lastname: Law
authors.forename: William M
authors.initials: WM
authors.lastname: Wara
authors.forename: Richard J
authors.initials: RJ
authors.lastname: O'Donnell
authors.forename: Katherine K
authors.initials: KK
authors.lastname: Matthay
authors.forename: Steven G
authors.initials: SG
authors.lastname: DuBois
authors.forename: Robert
authors.initials: R
authors.lastname: Goldsby
authors.forename: Daphne A
authors.initials: DA
authors.lastname: Haas-Kogan
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000379:methods; D018450:Disease Progression; D005260:Female; D018222:Desmoid Tumors / Q000188:drug therapy / Q000532:radiotherapy* / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy / Q000532:radiotherapy* / Q000601:surgery; D016879:Salvage Therapy / Q000379:methods; D055815:Young Adult