International Ophthalmology

, Volume 34, Issue 4, pp 999–1005 | Cite as

Optic nerve glioma: an update

  • Akshay Gopinathan NairEmail author
  • Rima S. Pathak
  • Veena R. Iyer
  • Rashmin A. Gandhi
Review Paper


Optic nerve glioma is the most common optic nerve tumour. However, it has an unpredictable natural history. The treatment of optic nerve gliomas has changed considerably over the past few years. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas. The treatment of optic nerve glioma requires a multi-disciplinary approach where all treatment options may have to be implemented in a highly individualized manner. The aim of this review article is to present current diagnostic and treatment protocols for optic nerve glioma.


Optic nerve glioma Proptosis Neurofibromatosis Tumour 



We would like to acknowledge the contribution of Sumedh S Hoskote, MD; Mayo Clinic, Rochester, MN for the assistance provided and Veena Olma Noronha, MD; VRR Scans, Chennai, India for contributing the radiological images.

Conflict of interest



  1. 1.
    Wilhelm H (2009) Primary optic nerve tumours. Curr Opin Neurol 22(1):11–18PubMedCrossRefGoogle Scholar
  2. 2.
    Dutton JJ (1994) Gliomas of the anterior visual pathway. Surv Ophthalmol 38:427–452PubMedCrossRefGoogle Scholar
  3. 3.
    Miller NR (2004) Primary tumours of the optic nerve and its sheath. Eye (Lond). 18(11):1026–1037PubMedCrossRefGoogle Scholar
  4. 4.
    Cummings TJ, Provenzale JM, Hunter SB, Friedman AM, Klintworth GK, Bigner SH et al (2000) Gliomas of the optic nerve: histological, immunohistochemical (MIB-1 and p53), and MRI analysis. Acta Neuropathol 99:563–570PubMedCrossRefGoogle Scholar
  5. 5.
    Sharma A, Mohan K, Saini JS (1990) Haemorrhagic changes in pilocytic astrocytoma of the optic nerve. Orbit 9:29–33CrossRefGoogle Scholar
  6. 6.
    Shapey J, Danesh-Meyer HV, Kaye AH (2011) Diagnosis and management of optic nerve glioma. J Clin Neurosci 18(12):1585–1591PubMedCrossRefGoogle Scholar
  7. 7.
    Listernick R, Ferner RE, Liu GT et al (2007) Optic pathway gliomas in neurofibromatosis-1: controversies and recommendations. Ann Neurol 61:189–198PubMedCrossRefGoogle Scholar
  8. 8.
    Czyzyk E, Józwiak S, Roszkowski M et al (2003) Optic pathway gliomas in children with and without neurofibromatosis 1. J Child Neurol 18:471–478PubMedCrossRefGoogle Scholar
  9. 9.
    Grill J, Laithier V, Rodriguez D et al (2000) When do children with optic pathway tumours need treatment? An oncological perspective in 106 patients treated in a single centre. Eur J Pediatr 159:692–696PubMedCrossRefGoogle Scholar
  10. 10.
    Balcer LJ, Liu GT, Heller G et al (2001) Visual loss in children with neurofibromatosis type 1 and optic pathway gliomas: relation to tumor location by magnetic resonance imaging. Am J Ophthalmol 131:442–445PubMedCrossRefGoogle Scholar
  11. 11.
    Dutton JJ (1991) Optic nerve gliomas and meningiomas. Neurol Clin 9:163–177PubMedGoogle Scholar
  12. 12.
    Chung EM, Specht CS, Schroeder JW (2007) From the archives of the AFIP: pediatric orbit tumors and tumorlike lesions: neuroepithelial lesions of the ocular globe and optic nerve. Radiographics 27(4):1159–1186PubMedCrossRefGoogle Scholar
  13. 13.
    Becker M, Masterson K, Delavelle J, Viallon M, Vargas MI, Becker CD (2010) Imaging of the optic nerve. Eur J Radiol 74(2):299–313PubMedCrossRefGoogle Scholar
  14. 14.
    Barkovich AJ (2005) Pediatric neuroimaging, 4th edn. Lippincott Williams & Wilkins, PhiladelphicGoogle Scholar
  15. 15.
    Weber AL, Caruso P, Sabates NR (2005) The optic nerve: radiologic, clinical, and pathologic evaluation. Neuroimaging Clin N Am 15(1):175–201PubMedCrossRefGoogle Scholar
  16. 16.
    Bianchi-Marzoli S, Brancato R (1994) Tumors of the optic nerve and chiasm. Curr Opin Ophthalmol 5:11–17PubMedCrossRefGoogle Scholar
  17. 17.
    Silva MM, Goldman S, Keating G et al (2000) Optic pathway hypothalamic gliomas in children under 3 years of age: the role of chemotherapy. Pediatr Neurosurg 33:151–158PubMedCrossRefGoogle Scholar
  18. 18.
    Packer RJ, Ater J, Allen J et al (1997) Carboplatin and vincristine for children with newly diagnosed progressive low-grade gliomas. J Neurosurg 86:747–754PubMedCrossRefGoogle Scholar
  19. 19.
    Packer RJ, Lange B, Ater J et al (1993) Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11:850–856PubMedGoogle Scholar
  20. 20.
    Lancaster DL, Hoddes JA, Michalski A et al (2003) Tolerance of nitrosourea-based multiagent chemotherapy regime for low-grade pediatric gliomas. J Neuro-Oncol 63:289–294CrossRefGoogle Scholar
  21. 21.
    Taveras JM, Mount LA, Wood EH (1956) The value of radiation therapy in the management of glioma of the optic nerves and chiasm. Radiology 66:518–528PubMedCrossRefGoogle Scholar
  22. 22.
    Montgomery AB, Griffin T, Parker RG et al (1977) Optic nerve glioma: the role of radiation therapy. Cancer 40:2079–2080PubMedCrossRefGoogle Scholar
  23. 23.
    Dororetz DE, Blitzer PH, Wang CC et al (1980) Management of glioma of the optic nerve and/or chiasm: an analysis of 20 cases. Cancer 45:1467–1471CrossRefGoogle Scholar
  24. 24.
    Tao ML, Barnes PD, Billett AL et al (1997) Childhood optic chiasm gliomas: radiographic response following radiotherapy and long-term clinical outcome. Int J Radiat Oncol Biol Phys 39(3):579–587PubMedCrossRefGoogle Scholar
  25. 25.
    Jenkin D, Angyalfi S, Becker L et al (1993) Optic glioma in children: surveillance, resection, or irradiation? Int J Radiat Oncol Biol Phys 25(2):215–225PubMedCrossRefGoogle Scholar
  26. 26.
    Pierce SM, Barnes PD, Loeffler JS et al (1990) Definitive radiation therapy in the management of symptomatic patients with optic glioma. Survival and long-term effects. Cancer 65(1):45–52PubMedCrossRefGoogle Scholar
  27. 27.
    Kovalic JJ, Grigsby PW, Shepard MJ et al (1990) Radiation therapy for gliomas of the optic nerve and chiasm. Int J Radiat Oncol Biol Phys 18(4):927–932PubMedCrossRefGoogle Scholar
  28. 28.
    Cappelli C, Grill J, Raquin M et al (1998) Long-term follow up of 69 patients treated for optic pathway tumours before the chemotherapy era. Arch Dis Child 79(4):334–338PubMedCentralPubMedCrossRefGoogle Scholar
  29. 29.
    Shrieve DC, Kooy HM, Tarbell NJ, Loeffler JS (1999) Fractionated stereotactic radiotherapy. In: Alexander E, Maciunas RJ (eds) Advanced neurosurgical navigation. Thieme Medical, Stuttgart, pp 451–454Google Scholar
  30. 30.
    Combs SE, Schulz-Ertner D, Moschos D et al (2005) Fractionated stereotactic radiotherapy of optic pathway gliomas: tolerance and long-term outcome. Int J Radiat Oncol Biol Phys 62(3):814–819PubMedCrossRefGoogle Scholar
  31. 31.
    Lacaze E, Kieffer V, Streri A et al (2003) Neuropsychological outcome in children with optic pathway tumours when first-line treatment is chemotherapy. Br J Cancer 89(11):2038–2044PubMedCentralPubMedCrossRefGoogle Scholar
  32. 32.
    Chadderton RD, West CG, Schuller S et al (1995) Radiotherapy in the treatment of low-grade astrocytomas. II. The physical and cognitive sequelae. Childs Nerv Syst 11(8):443–448 (erratum in: Childs Nerv Syst 1995 Dec; 11(12):715)Google Scholar
  33. 33.
    Brauner R, Malandry F, Rappaport R et al (1990) Growth and endocrine disorders in optic glioma. Eur J Pediatr 149(12):825–828PubMedCrossRefGoogle Scholar
  34. 34.
    Kestle JR, Hoffman HJ, Mock AR et al (1993) Moyamoya phenomenon after radiation for optic glioma. J Neurosurg 79(1):32–35PubMedCrossRefGoogle Scholar
  35. 35.
    Kortmann R-D, Timmermann B, Taylor RE et al (2003) Current and future strategies in radiotherapy of childhood low-grade glioma of the brain. Part I: treatment modalities of radiation therapy. Strahlenther Onkol 179:509–520PubMedCrossRefGoogle Scholar
  36. 36.
    Fouladi M, Wallace D, Langston JW et al (2003) Survival and functional outcome of children with hypothalamic/chiasmatic tumors. Cancer 97:1084–1092PubMedCrossRefGoogle Scholar
  37. 37.
    Spoor TC, Kennerdell JS, Martinez Z, Zorub D (1980) Malignant gliomas of the optic nerve pathways. Am J Ophthalmol 89:284–292PubMedGoogle Scholar
  38. 38.
    Danesh-Meyer HV, Savino PJ, Bilyk JR, Sergott RC (2005) Aggressive glioma of adulthood simulating ischemic optic neuropathy. Arch Ophthalmol 123:694–700PubMedCrossRefGoogle Scholar
  39. 39.
    Brodovsky S, ten Hove MW, Pinkerton RM, Ludwin SK, Smith RM (1997) An enhancing optic nerve lesion: malignant glioma of adulthood. Can J Ophthalmol 32:409–413PubMedGoogle Scholar
  40. 40.
    Gandhi RA, Nair AG (2011) Role of imaging in the management of neuro-ophthalmic disorders. Indian J Ophthalmol 59(2):111–116PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2014

Authors and Affiliations

  • Akshay Gopinathan Nair
    • 1
    Email author
  • Rima S. Pathak
    • 2
  • Veena R. Iyer
    • 3
  • Rashmin A. Gandhi
    • 1
  1. 1.Department of Neuro-OphthalmologySankara Nethralaya, A Unit of Medical Research FoundationChennaiIndia
  2. 2.Department of Radiation OncologyTata Memorial HospitalMumbaiIndia
  3. 3.Department of Diagnostic RadiologyUniversity of MinnesotaMinneapolisUSA

Personalised recommendations