Medscape is available in 5 Language Editions – Choose your Edition here.


Optic Nerve Sheath Meningioma Workup

  • Author: Mitchell V Gossman, MD; Chief Editor: Hampton Roy, Sr, MD  more...
Updated: May 18, 2016

Imaging Studies

See the list below:

  • Plain radiographs usually are not diagnostic, unless end-stage enlargement of the optic canal or hyperostosis is present.
  • CT scan with contrast is an excellent imaging technique for evaluation of optic nerve sheath meningioma (ONSM).
    • Thin slices (1.5-3 mm) must be taken to assess the extent of the tumor. ONSM is confined to the dura mater; therefore, it often appears as a distinct, fusiform thickening of the optic nerve. This enlargement may appear as localized or as an eccentric expansion of the optic nerve, and it occurs most commonly at the orbital apex.
    • ONSMs usually enhance homogeneously. In addition, linear, diffuse, or patchy calcifications within or along an optic nerve mass are commonly seen.[12]
    • Meningiomas surround the optic nerve. The diameter of the nerve is attenuated within the surrounding mass, giving a bull's eye appearance on coronal images and a tram track appearance on axial images.[12] (With optic nerve glioma, the nerve itself is expanded; there is no bull's eye appearance on coronal section, but a sagittal kinking is often seen.)
    • Although an MRI most readily shows the soft tissue tumor characteristics of ONSM, a CT scan better displays any associated bony hyperostosis.
  • Gadolinium-enhanced fat-suppression, T1-weighted pulse sequences allow visualization of meningiomas as a localized or tubular enlargement with significant contrast enhancement.
    • On most MRI pulse sequences, the tumor appears isointense as compared to the optic nerve and the brain tissue. Compared to the brain tissue, meningiomas may appear hypointense on T1-weighted images and hyperintense on T2-weighted images.
    • Other helpful MRI techniques include enhanced fat-suppression, T1-weighted images. Intraorbital extension of intracranial meningioma can be detected easily with contrast enhanced CT scan or MRI.
  • The exact knowledge of bony involvement in meningiomas is imperative because unresected meningiomatosis bone is one of the important factors that may contribute to recurrence.
    • EPM may be difficult to detect by radiologic evaluation. Plain radiographs may demonstrate hyperostosis, which may be present in up to 44% of patients with EPM.
    • Differential diagnosis includes all lesions that can be associated with focal hyperostosis (eg, osteomas, Paget disease, neurosarcoidosis, tuberculosis, lymphoma, fibrous dysplasia).
  • Standard tomography may demonstrate a thin layer of calcium deposit along the inner table of the hyperostosis bone with a superimposed lucency, which is thought to be dura mater. More invasive radiologic examinations, such as cerebral angiograms, are not of great value. Scintigraphic studies are of limited value.
  • CT scan in EPM often shows hyperostosis when present. Hyperostosis may involve the outer and inner tables with surface irregularity. With contrast, enhancement of the meninges may be seen in the area underlying the hyperostosis.
  • MRI without enhancement is not superior to CT scan. Both CT scan and MRI may show minimal parenchymal edema. Plaques are usually not visualized with either imaging technique.
    • Gadolinium may be helpful to demonstrate meningeal enhancement on T1-weighted sequences.
    • For suspected cases of juxtaorbital meningiomas, thinner slices of the orbit (3 mm) with T1-weighted MRI pulse sequence of the orbit are needed to improve the subtle contrast enhancement around and within the orbit.
    • Postcontrast fat-suppression, T1-weighted MRI images in axial and coronal planes also are useful because fat-suppression allows better visualization of the gray scale. At present, this technique is the best available imaging tool for the correct diagnosis of EPM.
      Meningioma of the orbit. Axial sequence on T1-weig Meningioma of the orbit. Axial sequence on T1-weighted MRI with gadolinium that shows enhancing lesion of the orbit causing proptosis and en plaque invagination laterally around the temporal pole and medially above the ethmoid sinus.
      Meningioma of the optic nerve sheath. Coronal sect Meningioma of the optic nerve sheath. Coronal section of T1-weighted MRI of the orbits that shows a left orbital mass lesion occupying most of the orbital lumen, diffusely enhancing with gadolinium.

Histologic Findings

Historically, the classification of meningiomas has been based upon cell shapes, cell patterns, and cell products. The macroscopic appearance of meningiomas may be hemispheric, bun-shaped, or globular, and they may vary in gross appearance as epithelial or mesenchymal. They usually are attached to the dura and invaginate into adjacent neural structures. Enveloped in a thin capsule derived from the adjacent meninges, they remain extraaxial and are separated easily from the brain or the spinal cord.

According to the World Health Organization (WHO) in 1993, 3 different types of meningiomas exist based on malignant behavior, as follows:

  • Benign (grade I) with a recurrence rate of 6.9%: Despite invasion of the adjacent bony structures, grade I meningiomas do not invade the brain parenchyma.
  • Atypical (grade II) with a recurrence rate of 34.6%: This type of meningioma shows frequent mitosis and an increased nuclear-cytoplasmic ratio.
  • Malignant (grades III and IV) with a recurrence rate of 72.7%: This type shows even greater mitosis, necrosis, and invasion of brain parenchyma.

Malignant transformation is rare. Originally, malignancy was seen in anaplastic tumors, but they may arise from any of the meningioma variants or atypical meningiomas. Papillary histopathology is associated with local aggressiveness and increased incidence of late distant metastasis. The papillary type is considered malignant by definition and is encountered more frequently in children.

Earlier classification schemes used the term angioblastic meningioma for what is now considered to be a hemangiopericytoma. This neoplasm is distinctly separate from a meningioma, and it shows extremely high propensity for recurrence and metastasis. Hemangiopericytoma is a sarcoma in the new WHO classification.

Growth type classification of meningiomas according to cell type is as follows:

  • Meningothelial growth is characterized by lobules of cells, marginated chromatin, and pseudoinclusions (ie, invagination of cell and nuclear membrane).
  • Fibroblastic growth is characterized by spindle-shaped cells in parallel interlacing bundles, intercellular collagen, and reticulin.
  • Transitional growth shows mixed features of the other 2 categories, and it commonly includes whorls and psammoma bodies usually not present in the other 2 types. Cell type is not indicative of expected behavior of the tumor.
Contributor Information and Disclosures

Mitchell V Gossman, MD Partner and Vice President, Eye Surgeons and Physicians, PA; Medical Director, Central Minnesota Surgical Center; Clinical Associate Professor, University of Minnesota Medical School

Mitchell V Gossman, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, Minnesota Medical Association, North American Neuro-Ophthalmology Society, Phi Beta Kappa

Disclosure: Nothing to disclose.


Sally B Zachariah, MD Associate Professor, Department of Neurology, University of South Florida College of Medicine; Director, Department of Neurology, Division of Strokes, Veteran Affairs Medical Center of Bay Pines

Sally B Zachariah, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Society of Neuroimaging

Disclosure: Partner received none from none for none.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew W Lawton, MD Neuro-Ophthalmology, Ochsner Health Services

Andrew W Lawton, MD is a member of the following medical societies: American Academy of Ophthalmology, Arkansas Medical Society, Southern Medical Association

Disclosure: Nothing to disclose.


Suzan Khoromi, MD Fellow, Pain and Neurosensory Mechanisms Branch, National Institute of Dental and Cranial Research, National Institutes of Health

Suzan Khoromi, MD is a member of the following medical societies: American Academy of Neurology, American Pain Society, and International Association for the Study of Pain

Disclosure: Nothing to disclose.

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

  1. Cushing H, Eisenhardt L. Meningiomas: their classification, regional behaviour, life history, and surgical end results. Springfield, Ill: Charles Thomas; 1938.

  2. Coke CC, Corn BW, Werner-Wasik M, Xie Y, Curran WJ Jr. Atypical and malignant meningiomas: an outcome report of seventeen cases. J Neurooncol. 1998 Aug. 39(1):65-70. [Medline].

  3. Menon G, Nair S, Sudhir J, Rao BR, Mathew A, Bahuleyan B. Childhood and adolescent meningiomas: a report of 38 cases and review of literature. Acta Neurochir (Wien). 2009 Mar. 151(3):239-44; discussion 244. [Medline].

  4. Harold Lee HB, Garrity JA, Cameron JD, Strianese D, Bonavolontà G, Patrinely JR. Primary optic nerve sheath meningioma in children. Surv Ophthalmol. 2008 Nov-Dec. 53(6):543-58. [Medline].

  5. Sheikh BY, Siqueira E, Dayel F. Meningioma in children: a report of nine cases and a review of the literature. Surg Neurol. 1996 Apr. 45(4):328-35. [Medline].

  6. Pompili A, Derome PJ, Visot A, Guiot G. Hyperostosing meningiomas of the sphenoid ridge--clinical features, surgical therapy, and long-term observations: review of 49 cases. Surg Neurol. 1982 Jun. 17(6):411-6. [Medline].

  7. Boschetti NV, Smith JL, Osher RH, Gass JD, Norton EW. Fluorescein angiography of optociliary shunt vessels. J Clin Neuroophthalmol. 1981 Mar. 1(1):9-30. [Medline].

  8. Sibony PA, Krauss HR, Kennerdell JS, Maroon JC, Slamovits TL. Optic nerve sheath meningiomas. Clinical manifestations. Ophthalmology. 1984 Nov. 91(11):1313-26. [Medline].

  9. Chacko JG, Schatz NJ, Glaser JS. Delayed optic nerve complications after proton beam irradiation. Ann Ophthalmol (Skokie). 2008 Fall-Winter. 40(3-4):166-70. [Medline].

  10. McCutcheon IE. The biology of meningiomas. J Neurooncol. 1996 Sep. 29(3):207-16. [Medline].

  11. Ing EB, Garrity JA, Cross SA, Ebersold MJ. Sarcoid masquerading as optic nerve sheath meningioma. Mayo Clin Proc. 1997 Jan. 72(1):38-43. [Medline].

  12. [Guideline] Zimmerman RD, Seidenwurm DJ, Davis PC, Brunberg JA, De La Paz RL, Dormont PD, et al. Orbits, vision, and visual loss. [online publication]. Reston (VA): American College of Radiology (ACR); 2006. [Full Text].

  13. Miller NR. New concepts in the diagnosis and management of optic nerve sheath meningioma. J Neuroophthalmol. 2006 Sep. 26(3):200-8. [Medline].

  14. Milker-Zabel S, Huber P, Schlegel W, Debus J, Zabel-du Bois A. Fractionated stereotactic radiation therapy in the management of primary optic nerve sheath meningiomas. J Neurooncol. 2009 Apr 1. [Medline].

  15. Arvold ND, Lessell S, Bussiere M, Beaudette K, Rizzo JF, Loeffler JS, et al. Visual Outcome and Tumor Control After Conformal Radiotherapy for Patients With Optic Nerve Sheath Meningioma. Int J Radiat Oncol Biol Phys. 2009 Apr 28. [Medline].

  16. Smee RI, Schneider M, Williams JR. Optic nerve sheath meningiomas--non-surgical treatment. Clin Oncol (R Coll Radiol). 2009 Feb. 21(1):8-13. [Medline].

  17. Mirimanoff RO, Dosoretz DE, Linggood RM, Ojemann RG, Martuza RL. Meningioma: analysis of recurrence and progression following neurosurgical resection. J Neurosurg. 1985 Jan. 62(1):18-24. [Medline].

  18. Barbaro NM, Gutin PH, Wilson CB, Sheline GE, Boldrey EB, Wara WM. Radiation therapy in the treatment of partially resected meningiomas. Neurosurgery. 1987 Apr. 20(4):525-8. [Medline].

  19. Glaholm J, Bloom HJ, Crow JH. The role of radiotherapy in the management of intracranial meningiomas: the Royal Marsden Hospital experience with 186 patients. Int J Radiat Oncol Biol Phys. 1990 Apr. 18(4):755-61. [Medline].

  20. Black PM. Hormones, radiosurgery and virtual reality: new aspects of meningioma management. Can J Neurol Sci. 1997 Nov. 24(4):302-6. [Medline].

  21. Greenberg HS. Meningiomas. Gilman S, Goldstein GW, Waxman SG. Neurobase. 1st ed. Arbor Publishing: San Diego; 1998.

  22. Grunberg SM, Weiss MH, Spitz IM, Ahmadi J, Sadun A, Russell CA, et al. Treatment of unresectable meningiomas with the antiprogesterone agent mifepristone. J Neurosurg. 1991 Jun. 74(6):861-6. [Medline].

  23. De Monte F. Current management of meningiomas. Oncology (Williston Park). 1995 Jan. 9(1):83-91, 96; discussion 96, 99-101. [Medline].

  24. Black P, Kathiresan S, Chung W. Meningioma surgery in the elderly: a case-control study assessing morbidity and mortality. Acta Neurochir (Wien). 1998. 140(10):1013-6; discussion 1016-7. [Medline].

  25. Milosevic MF, Frost PJ, Laperriere NJ, Wong CS, Simpson WJ. Radiotherapy for atypical or malignant intracranial meningioma. Int J Radiat Oncol Biol Phys. 1996 Mar 1. 34(4):817-22. [Medline].

  26. Akeyson EW, McCutcheon IE. Management of benign and aggressive intracranial meningiomas. Oncology (Williston Park). 1996 May. 10(5):747-56; discussion 756-9. [Medline].

  27. Donnell MS, Meyer GA, Donegan WL. Estrogen-receptor protein in intracranial meningiomas. J Neurosurg. 1979 Apr. 50(4):499-502. [Medline].

  28. Finn JE, Mount LA. Meningiomas of the tuberculum sellae and planum sphenoidale. A review of 83 cases. Arch Ophthalmol. 1974 Jul. 92(1):23-7. [Medline].

  29. Firsching RP, Fischer A, Peters R, Thun F, Klug N. Growth rate of incidental meningiomas. J Neurosurg. 1990 Oct. 73(4):545-7. [Medline].

  30. Kennerdell JS, Maroon JC, Malton M, Warren FA. The management of optic nerve sheath meningiomas. Am J Ophthalmol. 1988 Oct 15. 106(4):450-7. [Medline].

  31. Kinjo T, Mukawa J, Koga H, Shingaki T. An extensive cranial base meningioma extending bilaterally into Meckel's cave: case report. Neurosurgery. 1997 Mar. 40(3):615-7; discussion 617-8. [Medline].

  32. Kyritsis AP. Chemotherapy for meningiomas. J Neurooncol. 1996 Sep. 29(3):269-72. [Medline].

  33. Langford LA. Pathology of meningiomas. J Neurooncol. 1996 Sep. 29(3):217-21. [Medline].

  34. Lee AG, Woo SY, Miller NR, Safran AB, Grant WH, Butler EB. Improvement in visual function in an eye with a presumed optic nerve sheath meningioma after treatment with three-dimensional conformal radiation therapy. J Neuroophthalmol. 1996 Dec. 16(4):247-51. [Medline].

  35. Lloyd GA. Primary orbital meningioma: a review of 41 patients investigated radiologically. Clin Radiol. 1982 Mar. 33(2):181-7. [Medline].

  36. Mafee MF, Goodwin J, Dorodi S. Optic nerve sheath meningiomas. Role of MR imaging. Radiol Clin North Am. 1999 Jan. 37(1):37-58, ix. [Medline].

  37. Maor MH. Radiotherapy for meningiomas. J Neurooncol. 1996 Sep. 29(3):261-7. [Medline].

  38. Maroon JC, Kennerdell JS. Optic nerve sheath meningioma. Arch Neurol. 1981 May. 38(5):326. [Medline].

  39. Nakagawa H, Lusins JO. Biplane computed tomography of intracranial meningiomas with extracranial extension. J Comput Assist Tomogr. 1980 Aug. 4(4):478-83. [Medline].

  40. Russel DS, Rubinstein LJ. Russell and Rubinstein's Pathology of Tumors of the Nervous System. Bigner DD, McLendon RE, Bruner JM, eds. Hodder Headline Group. Vol. 1:1998.

  41. Schlehofer B, Blettner M, Wahrendorf J. Association between brain tumors and menopausal status. J Natl Cancer Inst. 1992 Sep 2. 84(17):1346-9. [Medline].

  42. Zimmerman RA. Imaging of intrasellar, suprasellar, and parasellar tumors. Semin Roentgenol. 1990 Apr. 25(2):174-97. [Medline].

Meningioma of the orbit. Axial sequence on T1-weighted MRI with gadolinium that shows enhancing lesion of the orbit causing proptosis and en plaque invagination laterally around the temporal pole and medially above the ethmoid sinus.
Meningioma of the optic nerve sheath. Coronal section of T1-weighted MRI of the orbits that shows a left orbital mass lesion occupying most of the orbital lumen, diffusely enhancing with gadolinium.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.