title: Management of optic chiasmatic/hypothalamic astrocytomas in children.
abstract: OBJECTIVE
  The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out those tumors involving the optic chiasm only (chiasmatic tumors) from those also involving the hypothalamus (chiasmatic/hypothalamic tumors). The purpose of this study was: (i) to analyze the outcomes of chiasmatic and chiasmatic/hypothalamic tumors separately; and (ii) to determine the appropriateness of recommending radical surgical resection for the chiasmatic/hypothalamic tumors.
  
  
  METHODS
  A retrospective chart review of all newly diagnosed tumors involving the optic chiasm from 1982-1996 at British Columbia's Children's Hospital was performed.
  
  
  RESULTS
  There were 32 patients less than 16 years of age, 14 with chiasmatic and 18 with chiasmatic/hypothalamic astrocytomas, with an average duration of follow-up of 5.8 years and 6.3 years, respectively. Ten of the patients with chiasmatic tumors and none with chiasmatic/hypothalamic tumors had neurofibromatosis I. Thirteen of the 14 chiasmatic tumors were managed with observation only, and none had progression requiring active intervention. For the chiasmatic/hypothalamic tumors, eight patients had subtotal resections (>95% resection), six had partial resections (50-95%), three had limited resections (<50%), and one had no surgery. There were fewer complications associated with the limited resections, especially with respect to hypothalamic dysfunction. There was no correlation between the extent of resection (subtotal, partial, or limited) and the time to tumor progression (average 18 months).
  
  
  CONCLUSIONS
  In conclusion, chiasmatic and chiasmatic/hypothalamic tumors are different entities, which should be separated out for the purposes of any study. For the chiasmatic/hypothalamic tumors, there was more morbidity and no prolongation of time to progression when radical resections were compared to more limited resections. Therefore, if surgery is performed, it may be appropriate to do a surgical procedure that strives only to provide a tissue diagnosis and to decompress the optic apparatus and/or ventricular system.
authors.affiliation: Department of Surgery, University of British Columbia and British Columbia's Children's Hospital, Vancouver, Canada.
authors.forename: Paul
authors.initials: P
authors.lastname: Steinbok
authors.forename: Stephen
authors.initials: S
authors.lastname: Hentschel
authors.forename: Per
authors.initials: P
authors.lastname: Almqvist
authors.forename: D Douglas
authors.initials: DD
authors.lastname: Cochrane
authors.forename: Kenneth
authors.initials: K
authors.lastname: Poskitt
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005910:Glioma / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D007223:Infant; D008297:Male; D009897:Optic Chiasm / Q000473:pathology; D019574:Optic Nerve Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome