title: Multidisciplinary management of refractory orbital rhabdomyosarcoma.
abstract: OBJECTIVE
  Combined chemotherapy and radiation therapy have improved the survival of children with primary orbital rhabdomyosarcoma, but recurrence or persistence of the local orbital tumor still occurs. There are no established guidelines for dealing with these uncommon patients, and the authors present a review of the combined method treatment and outcome of children with refractory primary orbital rhabdomyosarcoma.
  
  
  METHODS
  From clinical databases, 67 children with orbital rhabdomyosarcoma were identified. Seven (10%) of the 67 children had tumors refractory to combined chemotherapy and radiation therapy and underwent exenteration or eye-sparing tumor excision. Their clinical course and outcome were reviewed retrospectively.
  
  
  RESULTS
  No patient was lost to follow-up, which ranged from 3.2 to 11 years. Five (71%) of the seven children with refractory tumor are still alive at more than 3 years after surgery (3.2-11 years; mean, 6.9). In one of the two children who died, tumor extended beyond the operative margins at exenteration, and the other child died with regional metastasis within a month of exenteration.
  
  
  CONCLUSIONS
  Although more than 90% of children with orbital rhabdomyosarcoma respond to combined therapy by pediatric oncologists and radiotherapists, local orbital (salvage) surgery by ophthalmologists may be of value in the minority of children with refractory tumors. All of the five surviving children appear to be disease free.
authors.affiliation: Orbital Clinic, Moorfields Eye Hospital, London, England.
authors.forename: G E
authors.initials: GE
authors.lastname: Mannor
authors.forename: G E
authors.initials: GE
authors.lastname: Rose
authors.forename: P N
authors.initials: PN
authors.lastname: Plowman
authors.forename: J
authors.initials: J
authors.lastname: Kingston
authors.forename: J E
authors.initials: JE
authors.lastname: Wright
authors.forename: S J
authors.initials: SJ
authors.lastname: Vardy
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009918:Orbital Neoplasms / Q000601:surgery / Q000628:therapy*; D019233:Retreatment; D012208:Rhabdomyosarcoma / Q000601:surgery / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome