Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1997 Mar;76(3):259–263. doi: 10.1136/adc.76.3.259

Visual pathway glioma: an erratic tumour with therapeutic dilemmas

A Shuper 1, G Horev 1, L Kornreich 1, S Michowiz 1, R Weitz 1, R Zaizov 1, I Cohen 1
PMCID: PMC1717103  PMID: 9135269

Abstract

Accepted 12 September 1996


OBJECTIVE—Our experience in children with visual pathway glioma (VPG) was reviewed to delineate its clinical characteristics.
DESIGN—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.



Full Text

The Full Text of this article is available as a PDF (159.0 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alvord E. C., Jr, Lofton S. Gliomas of the optic nerve or chiasm. Outcome by patients' age, tumor site, and treatment. J Neurosurg. 1988 Jan;68(1):85–98. doi: 10.3171/jns.1988.68.1.0085. [DOI] [PubMed] [Google Scholar]
  2. Beyer R. A., Paden P., Sobel D. F., Flynn F. G. Moyamoya pattern of vascular occlusion after radiotherapy for glioma of the optic chiasm. Neurology. 1986 Sep;36(9):1173–1178. doi: 10.1212/wnl.36.9.1173. [DOI] [PubMed] [Google Scholar]
  3. Borit A., Richardson E. P., Jr The biological and clinical behaviour of pilocytic astrocytomas of the optic pathways. Brain. 1982 Mar;105(Pt 1):161–187. doi: 10.1093/brain/105.1.161. [DOI] [PubMed] [Google Scholar]
  4. Dutton J. J. Gliomas of the anterior visual pathway. Surv Ophthalmol. 1994 Mar-Apr;38(5):427–452. doi: 10.1016/0039-6257(94)90173-2. [DOI] [PubMed] [Google Scholar]
  5. Epstein M. A., Packer R. J., Rorke L. B., Zimmerman R. A., Goldwein J. W., Sutton L. N., Schut L. Vascular malformation with radiation vasculopathy after treatment of chiasmatic/hypothalamic glioma. Cancer. 1992 Aug 15;70(4):887–893. doi: 10.1002/1097-0142(19920815)70:4<887::aid-cncr2820700427>3.0.co;2-9. [DOI] [PubMed] [Google Scholar]
  6. Gajjar A., Heideman R. L., Kovnar E. H., Langston J. A., Sanford R. A., Douglass E. C., Jenkins J. J., Horowitz M. E., Kun L. E. Response of pediatric low grade gliomas to chemotherapy. Pediatr Neurosurg. 1993 May-Jun;19(3):113–120. doi: 10.1159/000120714. [DOI] [PubMed] [Google Scholar]
  7. Hoffman H. J., Humphreys R. P., Drake J. M., Rutka J. T., Becker L. E., Jenkin D., Greenberg M. Optic pathway/hypothalamic gliomas: a dilemma in management. Pediatr Neurosurg. 1993 Jul-Aug;19(4):186–195. doi: 10.1159/000120729. [DOI] [PubMed] [Google Scholar]
  8. Hoyt W. F., Baghdassarian S. A. Optic glioma of childhood. Natural history and rationale for conservative management. Br J Ophthalmol. 1969 Dec;53(12):793–798. doi: 10.1136/bjo.53.12.793. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Jenkin D., Angyalfi S., Becker L., Berry M., Buncic R., Chan H., Doherty M., Drake J., Greenberg M., Hendrick B. Optic glioma in children: surveillance, resection, or irradiation? Int J Radiat Oncol Biol Phys. 1993 Jan 15;25(2):215–225. doi: 10.1016/0360-3016(93)90342-s. [DOI] [PubMed] [Google Scholar]
  10. Kuenzle C., Weissert M., Roulet E., Bode H., Schefer S., Huisman T., Landau K., Boltshauser E. Follow-up of optic pathway gliomas in children with neurofibromatosis type 1. Neuropediatrics. 1994 Dec;25(6):295–300. doi: 10.1055/s-2008-1073043. [DOI] [PubMed] [Google Scholar]
  11. Lefkowitz I. B., Packer R. J., Sutton L. N., Siegel K. R., Bruce D. A., Evans A. E., Schut L. Results of the treatment of children with recurrent gliomas with lomustine and vincristine. Cancer. 1988 Mar 1;61(5):896–902. doi: 10.1002/1097-0142(19880301)61:5<896::aid-cncr2820610507>3.0.co;2-c. [DOI] [PubMed] [Google Scholar]
  12. Listernick R., Charrow J., Greenwald M. J., Esterly N. B. Optic gliomas in children with neurofibromatosis type 1. J Pediatr. 1989 May;114(5):788–792. doi: 10.1016/s0022-3476(89)80137-4. [DOI] [PubMed] [Google Scholar]
  13. Listernick R., Charrow J., Greenwald M., Mets M. Natural history of optic pathway tumors in children with neurofibromatosis type 1: a longitudinal study. J Pediatr. 1994 Jul;125(1):63–66. doi: 10.1016/s0022-3476(94)70122-9. [DOI] [PubMed] [Google Scholar]
  14. Lövblad K. O., Remonda L., Ozdoba C., Huber P., Schroth G. Dural ectasia of the optic nerve sheath in neurofibromatosis type 1: CT and MR features. J Comput Assist Tomogr. 1994 Sep-Oct;18(5):728–730. doi: 10.1097/00004728-199409000-00009. [DOI] [PubMed] [Google Scholar]
  15. North K., Cochineas C., Tang E., Fagan E. Optic gliomas in neurofibromatosis type 1: role of visual evoked potentials. Pediatr Neurol. 1994 Mar;10(2):117–123. doi: 10.1016/0887-8994(94)90043-4. [DOI] [PubMed] [Google Scholar]
  16. Packer R. J., Bilaniuk L. T., Cohen B. H., Braffman B. H., Obringer A. C., Zimmerman R. A., Siegel K. R., Sutton L. N., Savino P. J., Zackai E. H. Intracranial visual pathway gliomas in children with neurofibromatosis. Neurofibromatosis. 1988;1(4):212–222. [PubMed] [Google Scholar]
  17. Packer R. J., Lange B., Ater J., Nicholson H. S., Allen J., Walker R., Prados M., Jakacki R., Reaman G., Needles M. N. Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol. 1993 May;11(5):850–856. doi: 10.1200/JCO.1993.11.5.850. [DOI] [PubMed] [Google Scholar]
  18. Packer R. J., Sutton L. N., Bilaniuk L. T., Radcliffe J., Rosenstock J. G., Siegel K. R., Bunin G. R., Savino P. J., Bruce D. A., Schut L. Treatment of chiasmatic/hypothalamic gliomas of childhood with chemotherapy: an update. Ann Neurol. 1988 Jan;23(1):79–85. doi: 10.1002/ana.410230113. [DOI] [PubMed] [Google Scholar]
  19. Packer R. J., Vezina G. Pediatric glial neoplasms including brain-stem gliomas. Semin Oncol. 1994 Apr;21(2):260–272. [PubMed] [Google Scholar]
  20. Painter M. J., Chutorian A. M., Hilal S. K. Cerebrovasculopathy following irradiation in childhood. Neurology. 1975 Feb;25(2):189–194. doi: 10.1212/wnl.25.2.189. [DOI] [PubMed] [Google Scholar]
  21. Pollack I. F. Brain tumors in children. N Engl J Med. 1994 Dec 1;331(22):1500–1507. doi: 10.1056/NEJM199412013312207. [DOI] [PubMed] [Google Scholar]
  22. Pons M. A., Finlay J. L., Walker R. W., Puccetti D., Packer R. J., McElwain M. Chemotherapy with vincristine (VCR) and etoposide (VP-16) in children with low-grade astrocytoma. J Neurooncol. 1992 Oct;14(2):151–158. doi: 10.1007/BF00177619. [DOI] [PubMed] [Google Scholar]
  23. Rosenstock J. G., Packer R. J., Bilaniuk L., Bruce D. A., Radcliffe J. L., Savino P. Chiasmatic optic glioma treated with chemotherapy. A preliminary report. J Neurosurg. 1985 Dec;63(6):862–866. doi: 10.3171/jns.1985.63.6.0862. [DOI] [PubMed] [Google Scholar]
  24. Rossi L. N., Pastorino G., Scotti G., Gazocchi M., Maninetti M. M., Zanolini C., Chiodi A. Early diagnosis of optic glioma in children with neurofibromatosis type 1. Childs Nerv Syst. 1994 Sep;10(7):426–429. doi: 10.1007/BF00303606. [DOI] [PubMed] [Google Scholar]
  25. Wisoff J. H. Management of optic pathway tumors of childhood. Neurosurg Clin N Am. 1992 Oct;3(4):791–802. [PubMed] [Google Scholar]
  26. Wong J. Y., Uhl V., Wara W. M., Sheline G. E. Optic gliomas. A reanalysis of the University of California, San Francisco experience. Cancer. 1987 Oct 15;60(8):1847–1855. doi: 10.1002/1097-0142(19871015)60:8<1847::aid-cncr2820600829>3.0.co;2-e. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES