Meningioma, Sphenoid Wing Clinical Presentation

  • Author: Sally B Zachariah, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 27, 2010
 

History

  • Common symptoms include slowly developing unilateral exophthalmos and slight bulging of the bone in the temporal region.
  • Diffuse tumor infiltration of the orbital area may result in painless proptosis.
  • Transient ischemic attack-like presentation has been reported.
  • Variants of the clinical syndrome include the following:
    • Anosmia
    • Oculomotor palsy
    • Painful ophthalmoplegia (Tolosa-Hunt syndrome)
    • Blindness and optic atrophy in 1 eye, sometimes with papilledema of the other eye (Foster Kennedy syndrome)
    • Mental changes
    • Increased intracranial pressure
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Physical

Sphenoid wing meningiomas can be associated with various cranial nerve dysfunction resulting from foraminal encroachment of cranial nerves located at the skull base. Rarely, a bruit can be heard over a highly vascular tumor.

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Causes

  • Head trauma was suggested as a possible etiology for meningiomas, but recent prospective studies have shown no increased incidence of meningiomas in patients with head trauma.
  • An increased incidence of meningiomas has been reported in patients with neurofibromatosis type II, and increased incidence of meningiomas has been reported in patients with abnormalities of chromosome 22.
    • Deletions on chromosome 22 have been demonstrated in 40% of meningiomas. These deletions seem to be associated mainly with the terminal portions of the long arm of chromosome 22.
    • Loss of heterozygosity has been reported in at least 1 locus of chromosome 22 in 60-65% of patients with benign meningiomas. The incidence of abnormality on chromosome 22 increases in atypical and malignant meningiomas. The possible culprit may be a 664-kb gene on this locus coding for an N -acetylglucosaminyltransferase gene. This gene codes for an amino acid glycosyl transferase enzyme, and absence of this enzyme may lead to abnormal glycosylation of proteins and lipids, resulting in tumor formation.
    • Other chromosomal abnormalities have been reported in association with the following: chromosome arms 1p, 7p, 14p, 18q, and 6q, and chromosome 10.
  • Various viruses that have been described in association with primary brain tumors, including SV-40, adenovirus, and papovavirus, have been identified in meningiomas.
  • Hormonal factors, namely estrogen and progesterone, have been implicated in the pathophysiology of meningiomas. Both estrogen and progesterone receptors have been reported in a large majority of meningiomas, with a greater percentage of meningiomas expressing active progesterone receptors than estrogen receptors.
  • The beta-receptor beta subtype of PDGF has been identified in 100% of meningiomas in one study.
  • In vitro, PDGF appears to enhance cell proliferation of meningioma cells. The medium in which meningioma cells are grown in vitro has been shown to contain PDGF, and supplementary addition of PDGF to cultured meningioma cells appears to stimulate their growth.
  • Vascular endothelial growth factor also seems to play a role in the biology of meningiomas. The degree of peritumoral edema on T2-weighted MRI has been correlated directly with the degree of staining intensity of the meningioma in vitro.
  • Epidermal growth factor has been described in nearly 100% of meningiomas in tissue culture.
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Contributor Information and Disclosures
Author

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: none None None

Coauthor(s)

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.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

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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Coronal T1-weighted MRI with gadolinium enhancement of a sphenoid wing meningioma with some degree of encasement of bilateral cavernous sinuses.
T1-weighted MRI with gadolinium (coronal section) of same patient with sphenoid wing meningioma. A better visualization of en plaque growth of the meningioma along the convexity of the cerebral hemisphere on the left side is seen, in addition to better illustration of intracavernous carotid arteries bilaterally and en plaque growth of meningioma inferiorly and laterally around both temporal lobes.
T1-weighted gadolinium enhanced (sagittal section) of same patient with meningioma of the sphenoid wing.
 
 
 
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