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Optic Nerve Sheath Meningioma

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Adult CNS Radiation Oncology

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Abstract

Optic nerve sheath meningiomas (ONSM) are rare, and usually benign, tumors involving meninges surrounding the optic pathway. They have a higher incidence in middle-aged females. Diagnosis is heavily reliant on magnetic resonance imaging (MRI). ONSM can be subclassified as primary ONSM (pONSM) and secondary ONSM (sONSM). ONSM, in general, exhibit slowly progressive enlargement, which invariably leads to visual symptoms and/or vision loss. Although surgery may have a role in sONSM, it commonly leads to postoperative blindness with pONSM. Radiotherapy (RT) remains the mainstay of treatment for this condition. Highly conformal RT techniques such as stereotactic fractionated RT (SFRT) have been shown to be effective in retaining functional vision, with low treatment-related morbidity. At fractionated doses of 50–54 Gy, almost all patients achieve local control, and 80% or more of patients report vision improvement or stabilization. In this chapter, we will review the clinical findings and the treatment recommendations for this condition.

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References

  1. Ostrom QT, Gittleman H, Fulop J, et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2008–2012. Neuro Oncol. 2015;17(Suppl 4):iv1–iv62.

    Google Scholar 

  2. Whittle IR, Smith C, Navoo P, et al. Meningiomas. Lancet. 2004;363(9420):1535–43.

    Google Scholar 

  3. Dutton JJ. Optic nerve sheath meningiomas. Surv Ophthalmol. 1992;37(3):167–83.

    Google Scholar 

  4. Sibony PA, Krauss HR, Kennerdell JS, et al. Optic nerve sheath meningiomas. Clinical manifestations. Ophthalmology. 1984;91(11):1313–26.

    Google Scholar 

  5. Vernooij MW, Ikram MA, Tanghe HL, et al. Incidental findings on brain MRI in the general population. N Engl J Med. 2007;357(18):1821–8.

    Google Scholar 

  6. Berman D, Miller NR. New concepts in the management of optic nerve sheath meningiomas. Ann Acad Med Singap. 2006;35(3):168–74.

    Google Scholar 

  7. Frisen L, Royt WF, Tengroth BM. Optociliary veins, disc pallor and visual loss. A triad of signs indicating spheno-orbital meningioma. Acta Ophthalmol. 1973;51(2):241–9.

    Google Scholar 

  8. Hollenhorst RW Jr, Hollenhorst RW Sr, MacCarty CS. Visual prognosis of optic nerve sheath meningiomas producing shunt vessels on the optic disk: the Hoyt-Spencer syndrome. Trans Am Ophthalmol Soc. 1977;75:141–63.

    Google Scholar 

  9. Carrasco JR, Penne RB. Optic nerve sheath meningiomas and advanced treatment options. Curr Opin Ophthalmol. 2004;15(5):406–10.

    Google Scholar 

  10. Jakobiec FA, Depot MJ, Kennerdell JS, et al. Combined clinical and computed tomographic diagnosis of orbital glioma and meningioma. Ophthalmology. 1984;91(2):137–55.

    Google Scholar 

  11. Wright JE, McNab AA, McDonald WI. Primary optic nerve sheath meningioma. Br J Ophthalmol. 1989;73(12):960–6.

    Google Scholar 

  12. Saeed P, Rootman J, Nugent RA, et al. Optic nerve sheath meningiomas. Ophthalmology. 2003;110(10):2019–30.

    Google Scholar 

  13. Samples JR, Robertson DM, Taylor JZ, et al. Optic nerve meningioma. Ophthalmology. 1983;90(12):1591–4.

    Google Scholar 

  14. Probst C, Gessaga E, Leuenberger AE. Primary meningioma of the optic nerve sheaths: case report. Ophthalmologica. 1985;190(2):83–90.

    Google Scholar 

  15. Kanamalla US. The optic nerve tram-track sign. Radiology. 2003;227(3):718–9.

    Google Scholar 

  16. Gala F. Magnetic resonance imaging of optic nerve. Indian J Radiol Imaging. 2015;25(4):421–38.

    Google Scholar 

  17. Klingenstein A, Haug AR, Miller C, et al. Ga-68-DOTA-TATE PET/CT for discrimination of tumors of the optic pathway. Orbit. 2015;34(1):16–22.

    Google Scholar 

  18. Chandra P, Purandare N, Shah S, et al. Somatostatin receptor SPECT/CT using 99mTc Labeled HYNIC-TOC aids in diagnosis of primary optic nerve sheath meningioma. Indian J Nucl Med. 2017;32(1):63–5.

    Google Scholar 

  19. Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016;131(6):803–20.

    Google Scholar 

  20. Ragel BT, Jensen RL. Molecular genetics of meningiomas. Neurosurg Focus. 2005;19(5):E9.

    Google Scholar 

  21. Liu Y, Pang JC, Dong S, et al. Aberrant CpG island hypermethylation profile is associated with atypical and anaplastic meningiomas. Hum Pathol. 2005;36(4):416–25.

    Google Scholar 

  22. Egan RA, Lessell S. A contribution to the natural history of optic nerve sheath meningiomas. Arch Ophthalmol. 2002;120(11):1505–8.

    Google Scholar 

  23. Schick U, Jung C, Hassler WE. Primary optic nerve sheath meningiomas: a follow-up study. Cent Eur Neurosurg. 2010;71(3):126–33.

    Google Scholar 

  24. Wright JE, Call NB, Liaricos S. Primary optic nerve meningioma. Br J Ophthalmol. 1980;64(8):553–8.

    Google Scholar 

  25. Kennerdell JS, Maroon JC, Malton M, et al. The management of optic nerve sheath meningiomas. Am J Ophthalmol. 1988;106(4):450–7.

    Google Scholar 

  26. Alper MG. Management of primary optic nerve meningiomas. Current status—therapy in controversy. J Clin Neuroophthalmol. 1981;1(2):101–17.

    Google Scholar 

  27. Jeremic B, Pitz S. Primary optic nerve sheath meningioma: stereotactic fractionated radiation therapy as an emerging treatment of choice. Cancer. 2007;110(4):714–22.

    Google Scholar 

  28. Mark LE, Kennerdell JS, Maroon JC, et al. Microsurgical removal of a primary intraorbital meningioma. Am J Ophthalmol. 1978;86(5):704–9.

    Google Scholar 

  29. Schick U, Dott U, Hassler W. Surgical management of meningiomas involving the optic nerve sheath. J Neurosurg. 2004;101(6):951–9.

    Google Scholar 

  30. Turbin RE, Thompson CR, Kennerdell JS, et al. A long-term visual outcome comparison in patients with optic nerve sheath meningioma managed with observation, surgery, radiotherapy, or surgery and radiotherapy. Ophthalmology. 2002;109(5):890–9. discussion 9–900

    Google Scholar 

  31. Adeberg S, Welzel T, Rieken S, et al. Prior surgical intervention and tumor size impact clinical outcome after precision radiotherapy for the treatment of optic nerve sheath meningiomas (ONSM). Radiat Oncol. 2011;6:117.

    Google Scholar 

  32. Smith JL, Vuksanovic MM, Yates BM, et al. Radiation therapy for primary optic nerve meningiomas. J Clin Neuroophthalmol. 1981;1(2):85–99.

    Google Scholar 

  33. Mondon H, Hamard H, Sales J, et al. Role of radiotherapy in the treatment of meningioma of the optic nerve. Bull Soc Ophtalmol Fr. 1985;85(3):379–82.

    Google Scholar 

  34. Becker G, Jeremic B, Pitz S, et al. Stereotactic fractionated radiotherapy in patients with optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys. 2002;54(5):1422–9.

    Google Scholar 

  35. Brada M, Rajan B, Traish D, et al. The long-term efficacy of conservative surgery and radiotherapy in the control of pituitary adenomas. Clin Endocrinol. 1993;38(6):571–8.

    Google Scholar 

  36. Saeed P, Blank L, Selva D, et al. Primary radiotherapy in progressive optic nerve sheath meningiomas: a long-term follow-up study. Br J Ophthalmol. 2010;94(5):564–8.

    Google Scholar 

  37. Shrieve DC, Hazard L, Boucher K, et al. Dose fractionation in stereotactic radiotherapy for parasellar meningiomas: radiobiological considerations of efficacy and optic nerve tolerance. J Neurosurg. 2004;101(Suppl 3):390–5.

    Google Scholar 

  38. Tishler RB, Loeffler JS, Lunsford LD, et al. Tolerance of cranial nerves of the cavernous sinus to radiosurgery. Int J Radiat Oncol Biol Phys. 1993;27(2):215–21.

    Google Scholar 

  39. Mayo C, Martel MK, Marks LB, et al. Radiation dose-volume effects of optic nerves and chiasm. Int J Radiat Oncol Biol Phys. 2010;76(3 Suppl):S28–35.

    Google Scholar 

  40. Leavitt JA, Stafford SL, Link MJ, et al. Long-term evaluation of radiation-induced optic neuropathy after single-fraction stereotactic radiosurgery. Int J Radiat Oncol Biol Phys. 2013;87(3):524–7.

    Google Scholar 

  41. Richards JC, Roden D, Harper CS. Management of sight-threatening optic nerve sheath meningioma with fractionated stereotactic radiotherapy. Clin Exp Ophthalmol. 2005;33(2):137–41.

    Google Scholar 

  42. Phase II Trial of Observation for Low-Risk Meningiomas and of Radiotherapy for Intermediate- and High-Risk Meningiomas [RTOG 0539 Protocol Information]. Available from: https://www.rtog.org/ClinicalTrials/ProtocolTable/StudyDetails.aspx?study=0539.

  43. Emami B, Lyman J, Brown A, et al. Tolerance of normal tissue to therapeutic irradiation. Int J Radiat Oncol Biol Phys. 1991;21(1):109–22.

    Google Scholar 

  44. Parsons JT, Bova FJ, Mendenhall WM, et al. Response of the normal eye to high dose radiotherapy. Oncology (Williston Park). 1996;10(6):837–47. discussion 47–8, 51–2

    Google Scholar 

  45. Krishnan R, Kumar I, Kyle G, et al. Radiation retinopathy after fractionated stereotactic conformal radiotherapy for primary intraorbital optic nerve sheath meningioma. J Neuroophthalmol. 2007;27(2):143–4.

    Google Scholar 

  46. Subramanian PS, Bressler NM, Miller NR. Radiation retinopathy after fractionated stereotactic radiotherapy for optic nerve sheath meningioma. Ophthalmology. 2004;111(3):565–7.

    Google Scholar 

  47. Tsao MN, Hoyt WF, Horton J, et al. Improved visual outcome with definitive radiation therapy for optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys. 1999;45(3);324–5.

    Google Scholar 

  48. Andrews DW, Faroozan R, Yang BP, et al. Fractionated stereotactic radiotherapy for the treatment of optic nerve sheath meningiomas: preliminary observations of 33 optic nerves in 30 patients with historical comparison to observation with or without prior surgery. Neurosurgery. 2002;51(4):890–902. discussion 3–4

    Google Scholar 

  49. Pitz S, Becker G, Schiefer U, et al. Stereotactic fractionated irradiation of optic nerve sheath meningioma: a new treatment alternative. Br J Ophthalmol. 2002;86(11):1265–8.

    Google Scholar 

  50. Narayan S, Cornblath WT, Sandler HM, et al. Preliminary visual outcomes after three-dimensional conformal radiation therapy for optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys. 2003;56(2):537–43.

    Google Scholar 

  51. Baumert BG, Villa S, Studer G, et al. Early improvements in vision after fractionated stereotactic radiotherapy for primary optic nerve sheath meningioma. Radiother Oncol. 2004;72(2):169–74.

    Google Scholar 

  52. Schroeder TM, Yogeswaren ST, Augspurger ME, et al. Intensity modulated radiation therapy for optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys. 2004;60(1);S315.

    Google Scholar 

  53. Sitathanee C, Dhanachai M, Poonyathalang A, et al. Stereotactic radiation therapy for optic nerve sheath meningioma; an experience at Ramathibodi Hospital. J Med Assoc Thail. 2006;89(10):1665–9.

    Google Scholar 

  54. Arvold ND, Lessell S, Bussiere M, et al. Visual outcome and tumor control after conformal radiotherapy for patients with optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys. 2009;75(4):1166–72.

    Google Scholar 

  55. Milker-Zabel S, Huber P, Schlegel W, et al. Fractionated stereotactic radiation therapy in the management of primary optic nerve sheath meningiomas. J Neurooncol. 2009;94(3):419–24.

    Google Scholar 

  56. Smee RI, Schneider M, Williams JR. Optic nerve sheath meningiomas—non-surgical treatment. Clin Oncol (R Coll Radiol). 2009;21(1):8–13.

    Google Scholar 

  57. Liu D, Xu D, Zhang Z, et al. Long-term results of Gamma Knife surgery for optic nerve sheath meningioma. J Neurosurg. 2010;113(Suppl):28–33.

    Google Scholar 

  58. Lesser RL, Knisely JP, Wang SL, et al. Long-term response to fractionated radiotherapy of presumed optic nerve sheath meningioma. Br J Ophthalmol. 2010;94(5):559–63.

    Google Scholar 

  59. Marchetti M, Bianchi S, Milanesi I, et al. Multisession radiosurgery for optic nerve sheath meningiomas—an effective option: preliminary results of a single-center experience. Neurosurgery. 2011;69(5):1116–22. discussion 22–3

    Google Scholar 

  60. Solda F, Wharram B, Gunapala R, et al. Fractionated stereotactic conformal radiotherapy for optic nerve sheath meningiomas. Clin Oncol (R Coll Radiol). 2012;24(8):e106–12.

    Google Scholar 

  61. Abouaf L, Girard N, Lefort T, et al. Standard-fractionated radiotherapy for optic nerve sheath meningioma: visual outcome is predicted by mean eye dose. Int J Radiat Oncol Biol Phys. 2012;82(3):1268–77.

    Google Scholar 

  62. Paulsen F, Doerr S, Wilhelm H, et al. Fractionated stereotactic radiotherapy in patients with optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys. 2012;82(2):773–8.

    Google Scholar 

  63. Adams G, Roos DE, Crompton JL. Radiotherapy for optic nerve sheath meningioma: a case for earlier intervention? Clin Oncol (R Coll Radiol). 2013;25(6):356–61.

    Google Scholar 

  64. Brower JV, Amdur RJ, Kirwan J, et al. Radiation therapy for optic nerve sheath meningioma. Pract Radiat Oncol. 2013;3(3):223–8.

    Google Scholar 

  65. Moyal L, Vignal-Clermont C, Boissonnet H, et al. Results of fractionated targeted proton beam therapy in the treatment of primary optic nerve sheath meningioma. J Fr Ophtalmol. 2014;37(4):288–95.

    Google Scholar 

  66. Lahkola A, Salminen T, Raitanen J, et al. Meningioma and mobile phone use--a collaborative case-control study in five North European countries. Int J Epidemiol. 2008;37(6):1304–13.

    Google Scholar 

  67. Jackson A, Patankar T, Laitt RD. Intracanalicular optic nerve meningioma: a serious diagnostic pitfall. AJNR Am J Neuroradiol. 2003;24(6):1167–70.

    Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Radiation Oncology, National University Cancer Institute, National University Health System, Singapore, Singapore

    Balamurugan A. Vellayappan

  2. Department of Radiation Oncology, University of Washington, Seattle, WA, USA

    Lia M. Halasz, Yolanda D. Tseng & Simon S. Lo

  3. Department of Neurological Surgery, University of Washington, Seattle, WA, USA

    Lia M. Halasz & Simon S. Lo

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  1. Balamurugan A. Vellayappan
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  2. Lia M. Halasz
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  3. Yolanda D. Tseng
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  4. Simon S. Lo
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Corresponding author

Correspondence to Simon S. Lo .

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Editors and Affiliations

  1. Department of Radiation Oncology, Keck School of Medicine of USC, Norris Cancer Hospital, Los Angeles, California, USA

    Eric L. Chang

  2. Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota, USA

    Paul D. Brown

  3. Department of Radiation Oncology, University of Washington Medical Center, Seattle, Washington, USA

    Simon S. Lo

  4. Department of Radiation Oncology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada

    Arjun Sahgal

  5. Department of Radiation Oncology, Taussig Cancer Institute Cleveland Clinic, Cleveland, Ohio, USA

    John H. Suh

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Vellayappan, B.A., Halasz, L.M., Tseng, Y.D., Lo, S.S. (2018). Optic Nerve Sheath Meningioma. In: Chang, E., Brown, P., Lo, S., Sahgal, A., Suh, J. (eds) Adult CNS Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-42878-9_16

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  • DOI: https://doi.org/10.1007/978-3-319-42878-9_16

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