title: Local Resection and Brachytherapy for Primary Orbital Rhabdomyosarcoma: Outcome and Failure Pattern Analysis.
abstract: OBJECTIVE
  Survival in patients with orbital rhabdomyosarcoma (RMS) is excellent. Therefore, new local treatment modalities, such as brachytherapy, have been developed to minimize adverse events. Since 1990, patients with orbital RMS and a residual tumor after induction chemotherapy were eligible for resection and brachytherapy. Otherwise patients received external beam radiotherapy. In this study, the authors describe the outcome for 20 patients with primary orbital RMS. The aim was to assess risk factors for treatment failure in this single center cohort.
  
  
  METHODS
  In this retrospective cohort study, the authors reviewed imaging studies, surgery reports, histology reports, and radiotherapy plans in a multidisciplinary setting. The authors included 20 consecutive patients with orbital RMS, treated between 1990 and 2007, (median age: 7.4 years, range: 0.7-16.1; median follow up: 11.5 years).
  
  
  RESULTS
  After induction chemotherapy, 12 patients were treated with surgery and brachytherapy, 2 with external beam radiotherapy, and in 5 patients who achieved complete remission, local treatment was withheld. In 1 patient, brachytherapy was incorrectly withheld after delayed surgery. Seven patients relapsed (no local treatment, N = 2; surgery and brachytherapy, N = 2; external beam radiotherapy, N = 2; surgery only, N = 1). The authors found no patient, tumor, or treatment characteristics that predisposed for treatment failure. Ten-year-overall survival and event-free survival were 89% and 65%, respectively.
  
  
  CONCLUSIONS
  Overall survival in this cohort of orbital RMS patients was good, including surgery and brachytherapy as treatment modality for orbital RMS resulted in an effective local treatment approach with fewer adverse events than external beam radiotherapy. The authors could not identify factors predisposing for treatment failure.
authors.affiliation: *Department of Pediatric oncology, Emma Children's Hospital-Academic Medical Center (EKZ-AMC), Amsterdam; †Department of Ophthalmology, Orbital Center, ‡Department of Radiology, §Department of Radiation Oncology, ‖Department of Plastic, Reconstructive and Hand Surgery, and ¶Department of Pathology, Academic Medical Center (AMC), University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: Reineke A
authors.initials: RA
authors.lastname: Schoot
authors.forename: Peerooz
authors.initials: P
authors.lastname: Saeed
authors.forename: Nicole J
authors.initials: NJ
authors.lastname: Freling
authors.forename: Leo E C M
authors.initials: LE
authors.lastname: Blank
authors.forename: Bradley R
authors.initials: BR
authors.lastname: Pieters
authors.forename: Johan N B
authors.initials: JN
authors.lastname: van der Grient
authors.forename: Simon D
authors.initials: SD
authors.lastname: Strackee
authors.forename: Johannes
authors.initials: J
authors.lastname: Bras
authors.forename: Huib N
authors.initials: HN
authors.lastname: Caron
authors.forename: Johannes H M
authors.initials: JH
authors.lastname: Merks
mesh_terms: D000293:Adolescent; D001918:Brachytherapy / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D013508:Ophthalmologic Surgical Procedures / Q000379:methods*; D009915:Orbit / Q000000981:diagnostic imaging / Q000601:surgery; D009918:Orbital Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D015996:Survival Rate / Q000639:trends; D014057:Tomography, X-Ray Computed