title: Childhood optic chiasm gliomas: radiographic response following radiotherapy and long-term clinical outcome.
abstract: OBJECTIVE
  In children with chiasmal gliomas, radiation therapy can arrest progressive visual and neurologic impairment. We examined the radiographic response and clinical outcomes after irradiation.
  
  
  METHODS
  Forty-two children (median age at diagnosis, 6.6 years) with chiasmal gliomas were managed as follows: 11 asymptomatic patients with neurofibromatosis-1 (NF-1) were observed only; 2 patients, less than 3 years old, underwent surgery and chemotherapy to delay irradiation; and 29 patients with progressive disease received radiation with or without prior surgery or chemotherapy. Time to radiographic response, long-term tumor control and late sequelae were reviewed for the 29 irradiated patients.
  
  
  RESULTS
  The probability of at least 50% radiographic response at 24 months after irradiation was 18.1% and increased to 38.2% by 48 months and 45.9% by 60 months. By actuarial analysis, the median time for such radiographic response was 62 months. For the 29 irradiated patients, the 10-year freedom from progression and overall survival rates were 100% and 89%, respectively (median follow-up for surviving patients, 108 months). Stabilization or improvement in vision occurred in 81% of 26 evaluable irradiated patients.
  
  
  CONCLUSIONS
  Notable radiographic response may be observed years after irradiation. Radiation therapy provides excellent long-term tumor control and vision preservation or improvement in the majority of patients with progressive chiasmal gliomas.
authors.affiliation: Joint Center for Radiation Therapy, The Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
authors.forename: M L
authors.initials: ML
authors.lastname: Tao
authors.forename: P D
authors.initials: PD
authors.lastname: Barnes
authors.forename: A L
authors.initials: AL
authors.lastname: Billett
authors.forename: T
authors.initials: T
authors.lastname: Leong
authors.forename: D C
authors.initials: DC
authors.lastname: Shrieve
authors.forename: R M
authors.initials: RM
authors.lastname: Scott
authors.forename: N J
authors.initials: NJ
authors.lastname: Tarbell
mesh_terms: D000293:Adolescent; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003390:Cranial Nerve Neoplasms / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D018450:Disease Progression; D004700:Endocrine System Diseases / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D006801:Humans; D007223:Infant; D007859:Learning Disabilities / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D009897:Optic Chiasm / Q000000981:diagnostic imaging* / Q000473:pathology; D011832:Radiation Injuries / Q000000981:diagnostic imaging / Q000209:etiology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014786:Vision Disorders / Q000209:etiology