Optic Nerve Sheath Meningioma 

  • Author: Mitchell V Gossman, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 24, 2012
 

Background

In 1614, Felix Plater first described meningiomas at an autopsy. In 1938, Harvey Cushing introduced them as a separate category of extraparenchymal tumors.[1] Meningiomas are believed to arise from arachnoid cap cells, and they usually are attached to the dura. These tumors may arise from any location where meninges exist (eg, nasal cavity, paranasal sinuses, middle ear, mediastinum).

In children, the more common locations of meningiomas include the orbit, the temporal region, the foramen magnum, the tentorial region, the subfrontal base, the sellar region, and the ethmoidal air sinus. Such locations for meningiomas are rare in adults. Histologically, the meningothelial type is seen most frequently. As compared to adults, the tumors in children tend to be more aggressive in terms of growth rate, tumor size, propensity to undergo malignant changes, and recurrence rate.

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Pathophysiology

The term optic nerve sheath meningioma (ONSM) does not indicate a definite site of origin. ONSM may be either primary or secondary. Primary ONSMs arise from the cap cells of the arachnoid surrounding the intraorbital or, less frequently, the intracanalicular optic nerve. Secondary ONSM are extensions of intracranial meningioma into the orbit. Secondary ONSMs are much more common than primary ONSMs, but the unqualified term "optic nerve sheath meningioma" ordinarily refers to primary ONSM.

Another rare group of meningiomas consists of tumors that arise from ectopic arachnoid cells within the orbital cavity, either in the muscle cone or in the walls of the orbit. These ectopic, extradural meningiomas do not appear to have a connection to the optic nerve sheath or the optic canal and do not appear to originate intracranially. They probably arise from congenitally displaced nests of meningothelial cells along the orbital wall or within the muscle cone.

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Epidemiology

Frequency

United States

The frequency of meningiomas has been the topic of relatively few reports. Hospital-based brain tumor series indicate that the incidence is approximately 20% of all intracranial tumors; population-based studies indicate an overall incidence of 2.3 cases per 100,000.

International

Meningiomas account for approximately 13-19% of all intracranial tumors.

Mortality/Morbidity

In one series by Coke et al, the overall survival rate for all patients at 5 years and 10 years were 87% and 58%, respectively.[2]

Race

Variability has been shown in the prevalence of meningiomas among Caucasians, Africans, African Americans, and Asians. A greater incidence among Africans than among Caucasians exists.

Sex

Although intracranial tumors as a whole show a higher prevalence in males than in females, meningiomas have a 2:1 female-to-male ratio in Caucasians.

  • In one series of 517 patients with meningiomas at Brigham and Women's Hospital, the female-to-male ratio was 24:1.
  • In children, the male-to-female ratio is 2:1, with an average age at presentation of 10.1 years (range, 1-16 y).
  • In Africans, an equal gender ratio is evident.

Age

The incidence of meningiomas increases with age, 2-7 cases per 100,000 in women and 1-5 cases per 100,000 in men. Peak incidence is in the seventh decade in women and in the eighth decade in men.[3, 4]

  • Meningiomas rarely occur in infants.
  • Meningiomas rarely occur in children[4] and differ from those in adults and other childhood tumors. Incidence in children is estimated to be approximately 2%. In one retrospective series at the King Faisal Hospital from 1980-1993 that included a total of 318 patients with meningiomas, only 2.8% of patients were children aged 16 years or younger. Meningiomas made up only 2.2% of all CNS tumors seen in children.
  • In one series by Sheikh et al, the mean age of juvenile cases was 8 years and the mean age for adults was 50 years.[5] Mean ages reported in other series range from 31.7-43 years. The male-to-female ratio is 27:29, adding together all series. Neurofibromatosis occurs in 4.2-16% of cases.
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Contributor Information and Disclosures
Author

Mitchell V Gossman, MD  Partner and Vice President, Eye Surgeons and Physicians, St Cloud

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, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

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, and American Society of Neuroimaging

Disclosure: none None None

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.

Specialty Editor Board

Andrew W Lawton, MD  Medical Director of Neuro-Ophthalmology Service, Section of Ophthalmology, Baptist Eye Center, Baptist Health Medical Center

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

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor 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, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

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.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

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, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

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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.
 
 
 
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