title: Visual pathway glioma: an erratic tumour with therapeutic dilemmas.
abstract: OBJECTIVE
  Our experience in children with visual pathway glioma (VPG) was reviewed to delineate its clinical characteristics.
  
  
  METHODS
  The charts and imaging studies of 21 children with VPG who were followed up in our centre during the last 12 years were reviewed and summarised.
  
  
  RESULTS
  VPG accounted for 13.1% of all brain tumours treated during this period. Sixty two per cent of the children with VPG had neurofibromatosis type 1 (NF-1). Among these, more than 60% were detected as part of routine work up. In some cases decreasing visual function preceded the appearance of the VPG on imaging studies. Tumour growth rate was markedly unpredictable. All treatment modalities employed led to tumour shrinkage and stabilisation for a variable period, but none was successful in totally eradicating the tumour. Complications were less severe after chemotherapy compared with radiotherapy. Three children died, none with NF-1, with a globular hypothalamic/chiasmatic tumour and accompanying electrolyte abnormalities.
  
  
  CONCLUSIONS
  NF-1 is a favourable prognostic marker for VPG. Whenever possible a period of observation is necessary before treatment is initiated, during which time tumour size and visual function should be closely followed up; an untoward change in either of these is an indication for the start of treatment, preferably chemotherapy first. The combination of a globular hypothalamic/chiasmatic glioma and electrolyte abnormalities in a child without NF-1 are related to a poor prognosis.
authors.affiliation: Schneider Children's Medical Center of Israel, Petah Tiqva, Israel.
authors.forename: A
authors.initials: A
authors.lastname: Shuper
authors.forename: G
authors.initials: G
authors.lastname: Horev
authors.forename: L
authors.initials: L
authors.lastname: Kornreich
authors.forename: S
authors.initials: S
authors.lastname: Michowiz
authors.forename: R
authors.initials: R
authors.lastname: Weitz
authors.forename: R
authors.initials: R
authors.lastname: Zaizov
authors.forename: I J
authors.initials: IJ
authors.lastname: Cohen
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002330:Carmustine / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003390:Cranial Nerve Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D003609:Dactinomycin / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005910:Glioma / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000628:therapy; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D009897:Optic Chiasm; D009901:Optic Nerve Diseases / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D014750:Vincristine / Q000627:therapeutic use; D014793:Visual Cortex
chemical_list: D000903:Antibiotics, Antineoplastic; D000972:Antineoplastic Agents, Phytogenic; D003609:Dactinomycin; D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D002330:Carmustine