eMedicine Specialties > Ophthalmology > Optic Nerve

Meningioma, Optic Nerve Sheath

Author: Mitchell V Gossman, MD, Partner and Vice President, Eye Surgeons and Physicians, St Cloud
Coauthor(s): Sally B Zachariah, MD, Associate Professor, Department of Neurology, University of South Florida; Director, Department of Neurology, Division of Strokes, Veteran Affairs Medical Center of Bay Pines; Suzan Khoromi, MD, Fellow, Pain and Neurosensory Mechanisms Branch, National Institute of Dental and Cranial Research, National Institutes of Health
Contributor Information and Disclosures

Updated: Jul 5, 2007

Introduction

Background

In 1614, Felix Plater first described meningiomas at an autopsy. In 1938, Harvey Cushing introduced them as a separate category of extraparenchymal tumors. 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.

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.

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.1

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.

  • Meningiomas rarely occur in infants.
  • Meningiomas rarely occur in children 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.2 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.

Clinical

History

  • The characteristic profile of a compressive optic neuropathy, such as that caused by ONSM, is usually painless, chronic progressive visual loss that may be accompanied by proptosis.
  • Transient visual obscurations also have been reported; however, this phenomenon is not a specific feature, as it has been observed in patients with optic disc swelling resulting from other causes.
  • Age of presentation varies widely, ranging from 3-76 years across all series. In several series, the incidence of visual loss as the presenting feature was more common than proptosis, even though in some series with more ophthalmologic detail, the presentation of proptosis was greater than visual loss. Presentation with either symptom complex is about equal.
  • Cases may be bilateral (approximately 6%).
  • An important macroscopic variant of ONSM is the en plaque meningioma (EPM), which is not greatly raised above the level of dura mater.

    • This type of meningioma grows within the meningeal sheath and expands the meninges without forming large exophytic masses. It is prone to invade adjacent bone with accompanying hyperostosis.
    • This group of meningiomas essentially infiltrates the brain and the orbit, mainly via natural apertures (eg, foramina, fissures) and through the passageway of perforating blood vessels, which are increased in number.
  • Hyperostosis is present in 44% of cases, but it is not a specific finding. EPM typically occurs at the base of the skull, especially within the sphenoid ridge. Clinical presentation depends on the site or extent of the tumor. Patients with juxtaorbital EPM often present with visual complaints. In a series in 1982, Pompili et al reported 33 of 49 cases of EPMs in a series of hyperostosis meningiomas of the sphenoid ridge.3 Clinical symptoms were not different between the various histopathological types (ie, no difference was seen clinically between EPM and the globular type of hyperostosis meningiomas of the sphenoid).
  • Common symptoms include the following: 

    • Exophthalmos (83%) 
    • Decreased visual acuity (38%) 
    • Headache (36%) 
    • Ptosis diplopia (21%)
  • Common neurologic complaints include the following:
    • Unilateral optic atrophy or subatrophy (29%) 
    • Decreased visual acuity (24%) 
    • Oculomotor disturbance (21%)
  • Hyperostosis and/or endostosis are common with meningiomas. Hyperostotic meningiomas are viewed as disease of the bone. Globular meningiomas, which are associated with bony reaction and invasion, are referred to as en masse (global) meningiomas.

Physical

  • The presentation of ONSMs largely depends on whether they arise from the orbit, within the optic canal, or intracranially. Notwithstanding, the classic triad of ONSM is visual loss, optic atrophy, and optociliary shunt vessels.
  • The characteristic symptom and sign of ONSM is very slow visual loss with preservation of the central visual field for years.
  • The presence of an optociliary venous shunt on the disc, if associated with disc pallor or visual loss, is suggestive but not pathognomonic of ONSM. These vessels are more appropriately termed retinochoroid venous collaterals and arise from chronic compression of the central retinal vein. These blood vessels shunt blood into choroidal venous circulation. Optociliary shunts result from meningiomas arising from the optic nerve sheath or from spheno-orbital meningiomas. They can occur with other types of tumors (eg, optic nerve gliomas) and chronic papilledema, but they occur most frequently from old central retinal vein occlusion.
  • Optic disc pallor and swelling have been reported equally in different series. If there is disc edema, the tumor is at least partially intraorbital.
  • Disturbance of ocular motility was mainly reported by Sibony et al in one series of 47 patients.4

Causes

A common etiology for meningiomas is radiation exposure in the range of 132-315 roentgens, which is equivalent to the rad dose of 1-3 Gy. Characteristics of radiation-induced meningiomas include an average latent period of 36-38 years for patients who were exposed to low-dose radiation to the head, whereas patients who develop meningiomas after exposure to high-dose radiation may show signs as early as 5 years postradiation.

  • In general, radiation-induced meningiomas occur more frequently over the convexities (ie, in about 80% of cases). They have a more frequent recurrence rate, and they exhibit malignant behavior that is also indicated histologically by hypercellularity and pleomorphism.
  • Head trauma used to be considered a possible risk factor, but recent large studies do not support this association.
  • Another factor that has been studied is hereditary predisposition.

    • Loss of DNA on chromosome 22 has been shown in 40% of meningiomas.
    • In cytogenetic studies, genetic abnormalities at the level of chromosome 22 are seen frequently in meningiomas with the loss of a copy of chromosome 22 as the most commonly reported abnormality.
    • Monosomy of chromosome 22 has been reported to occur in 70-80% of meningiomas.
    • Abnormalities of chromosome 22 have been associated with type II neurofibromatosis.
  • Hormonal factors, such as estrogen and progesterone, have been studied extensively as risk factors for meningiomas because of the striking predominance of meningiomas in women. Other evidence to substantiate the implication of gender-specific hormones comes from data showing increased growth of meningiomas during pregnancy and size changes with menses. Initially, interest was focused on estrogen because it had been reported in one comprehensive review by McCutcheon that 30% of meningiomas have estrogen receptors; however, no further studies were conducted, and interest became focused on other hormones (ie, progesterone).5
    • The progesterone receptor is the most likely candidate as an etiology for meningiomas. Progesterone receptors have been shown to be expressed in 81% of women and in 40% of men with meningiomas. Other studies indicate that progesterone binds to meningiomas in 50-100% of tested specimens; however, most reports show binding in the higher end of this range. No relation has been found between progesterone receptor status and age, sex, tumor location, or menopausal state. These findings have prompted researchers to develop antiprogesterone medications, such as mifepristone (RU-486), which appear to inhibit tumor growth in vitro and in vivo.
    • Androgen receptors have been found in 40-100% of meningiomas studied in several studies, but their receptor expression is variable, making them less likely candidates in the pathophysiology of meningiomas. Meningiomas vary in expression of receptors for other hormones (eg, epidermal growth factor [EGF], platelet derived growth factor [PDGF], fibroblast growth factor), which makes them less likely candidates for oncogenesis of meningiomas. It has been suggested that the direct stimulatory effect of EGF on PDGF or PDGF itself may be partially responsible for angiogenesis and even oncogenesis in meningiomas. PDGF is a particularly attractive candidate because it has structural homology with the product of c-sis oncogene on chromosome 22. Infectious agents that have been associated with meningiomas include simian vacuolating virus 40 (SV-40) and adenovirus.

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References
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Further Reading

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Keywords

perioptic meningioma, optic nerve sheath meningioma, ONSM, intracranial tumors, brain tumors, brain cancer, meningioma of the orbit, optic neuritis

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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; 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: 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.

Medical Editor

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.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

Managing Editor

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.

CME Editor

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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