title: Orbital rhabdomyosarcoma in children: a favorable primary suitable for a less-invasive treatment strategy.
abstract: BACKGROUND
  Orbital rhabdomyosarcoma (ORMS) treatment is based on combination chemotherapy associated with best local therapy, sometimes surgery but more often radiation therapy. A retrospective single-center analysis was conducted to more clearly define the long-term outcome of patients with ORMS, to identify patients in whom aggressive first-line local therapy can be avoided.
  
  
  METHODS
  A total of 95 patients with localized parameningeal (PM) or nonparameningeal (NPM) ORMS, treated at the Institut Curie between 1975 and 2010, were analyzed.
  
  
  RESULTS
  Median age at diagnosis was 6 years (range, 8 mo to 19.5 y), and median follow-up was 8.5 years (range, 7 mo to 24 y). A total of 25 patients presented PM extension. Radiation therapy was part of primary therapy for 78 patients. Five-year event-free survival and overall survival rates were 65.4%±5.2% and 85.6%±3.9%, respectively. On multivariate analysis, initial tumor size was identified as a significant prognostic factor. Event-free survival was similar for PM and NPM tumors (60.3%±10.4% vs. 62.7%±5.9%, P=0.57), whereas there was a trend for overall survival to be better for NPM tumors (90%±3.9% vs. 72.7%±9.6%, P=0.07).
  
  
  CONCLUSIONS
  Localized ORMS has a favorable outcome despite the current trend toward less aggressive and more limited indications of local therapy. Patients with a favorable pattern of strictly ORMS can be treated without first-line radiation therapy.
authors.affiliation: Departments of *Pediatric, Adolescent and Young Adult Oncology †Radiology ‡Biostatistics §Radiotherapy ¶Pathology ∥Ophthalmology, Institut Curie, Paris, France.
authors.forename: Helene
authors.initials: H
authors.lastname: Boutroux
authors.forename: Cecile
authors.initials: C
authors.lastname: Cellier
authors.forename: Véronique
authors.initials: V
authors.lastname: Mosseri
authors.forename: Sylvie
authors.initials: S
authors.lastname: Helfre
authors.forename: Christine
authors.initials: C
authors.lastname: Levy
authors.forename: Laurence
authors.initials: L
authors.lastname: Desjardins
authors.forename: Corine
authors.initials: C
authors.lastname: Plancher
authors.forename: Paul
authors.initials: P
authors.lastname: Freneaux
authors.forename: Jean
authors.initials: J
authors.lastname: Michon
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D059248:Chemoradiotherapy / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D003609:Dactinomycin / Q000627:therapeutic use; D018572:Disease-Free Survival; D015251:Epirubicin / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D007223:Infant; D009367:Neoplasm Staging; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy*; D011379:Prognosis; D011877:Radionuclide Imaging; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D003609:Dactinomycin; D015251:Epirubicin; D014750:Vincristine; D003520:Cyclophosphamide; D007069:Ifosfamide