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Tumors of the Optic Nerve Initiative


Frequently Asked Questions

  1. What is a primary optic nerve tumor?
  2. What is a secondary optic nerve tumor?
  3. What are the signs and symptoms of an optic nerve glioma?
  4. What is the treatment for optic nerve glioma?
  5. What is an optic nerve sheath meningioma?
  6. What are the signs and symptoms of an optic nerve sheath meningioma?
  7. What is the treatment for optic nerve sheath meningioma?
  8. What is the purpose of the Tumors of the Optic Nerve Initiative and the Optic Nerve Center?
  9. What can the Optic Nerve Center do for me?
  10. What are the strengths of the Optic Nerve Center?
  11. What is the Holden Comprehensive Cancer Center?
  12. Are the departments and faculty of the Optic Nerve Center well known?
  13. What are the advantages of coming to the Center for optic pathway gliomas?
  14. What is the advantage of coming to the Center for an optic nerve meningioma?

What is a primary optic nerve tumor?

A primary optic nerve tumor is one that arises from the optic nerve itself or its covering (the meninges). The two most primary common optic nerve tumors are optic glioma and optic nerve meningioma.

What is a secondary optic nerve tumor?

A secondary optic nerve tumor arises from the brain or adjacent structures but compresses the optic nerve and produces symptoms.

What are the signs and symptoms of an optic nerve glioma?

Optic glioma is typically a tumor of childhood. It may be associated with an inherited disorder known as neurofibromatosis I. The symptoms of optic glioma are usually related to visual loss due to compression of the optic nerve. Bulging of the eye (proptosis), double vision (ocular misalignment), and drooping of the eyelid (ptosis) may also occur. Patients with gliomas that involve the optic nerves and optic pathway in the brain may have hydrocephalus ("water on the brain") or hormonal imbalance.

What is the treatment for optic nerve glioma?

Optic gliomas may be treated by observation alone, chemotherapy, radiation therapy or surgery.

What is an optic nerve sheath meningioma?

An optic nerve sheath meningioma is a tumor of the covering of the optic nerve (the meninges).

What are the signs and symptoms of an optic nerve sheath meningioma?

Optic nerve sheath meningioma is typically a tumor of adulthood and is seen more frequently in middle aged women. The symptoms of optic nerve sheath meningioma are usually related to visual loss due to compression of the optic nerve. Bulging of the eye (proptosis), double vision (ocular misalignment), and drooping of the eyelid (ptosis) may also occur.

What is the treatment for optic nerve sheath meningioma?

Optic nerve sheath meningiomas may be treated with observation alone, radiation therapy, or surgery.

What is the purpose of the Tumors of the Optic Nerve Initiative and the Optic Nerve Center?

The goal of the Optic Nerve Center is to provide high quality and cutting edge evaluation and treatment for patients with tumors affecting the optic nerve. The Center hopes to develop and study new and emerging treatments for optic nerve tumors and to serve as a national model for innovative and novel approaches to optic nerve tumors. The Center's purpose is to serve as a focal point and centerpiece of a multidisciplinary approach to these tumors.

What can the Optic Nerve Center do for me?

A multidisciplinary approach to these tumors enhances patient care. Patients benefit from a coordinated clinical service that is "under one roof". The disciplines that currently participate in the care of these patients include:

  • Neuro-ophthalmology
  • Orbit and oculoplastic surgery
  • Neurosurgery
  • Neuroradiology
  • Adult and pediatric neuro-oncology
  • Radiation therapy

What are the strengths of the Optic Nerve Center?

The Optic Nerve Center offers world class service in multiple disciplines.

  • Radiation therapy. New and emerging modalites in radiation therapy allow more advanced treatment of optic nerve lesions and spare surrounding brain, retina, and contralateral optic nerve. These modalities include stereotactic radiotherapy and radiosurgery and conformal intensity modulated radiotherapy.
  • Pediatric and adult neuro-oncology. The oncologists at the Holden Cancer Center have tremendous experience with chemotherapy and the medical treatment of tumors. There is increasing evidence on the efficacy of chemotherapy in optic nerve gliomas. There is enhanced potential for clinical trials on chemotherapy and hormonal therapy in meningiomas and other tumors of the optic nerve.
  • Neurosurgery. The neurosurgeons have developed new and improved image guided procedures and techniques. The Center provides improved coordination and access to care and a multispecialty team approach to tumors.
  • Orbit and oculoplastic surgery. Tumors that involve the brain and the orbit benefit from a coordinated surgical plan. Improved cooperation and coordination with neurosurgery and newer advanced image guided biopsy procedures may improve surgical outcomes in patients with tumors of the optic nerve.
  • Neuro-ophthalmology. The Neuro-ophthalmology service is the focal point for diagnosis and referral of optic nerve tumors. The specialists in optic nerve function provide detailed quantitative evaluation and follow-up information for prognosis.The Neuro-ophthalmology services has unique expertise and novel optic nerve imaging and psychomotor techniques including:
    • Multifocal electroretinography (MERG)
    • Multifocal visual evoked potential (MVEP)
    • Motion perimetry
    • Pupil perimetry
    • Optical coherence tomography (OCT)
    • Orbital echography
    • 24 hour blood pressure measurements
    • Video fluorescein angiography
    • Retinal nerve fiber analysis

What is the Holden Comprehensive Cancer Center?

The Holden Comprehensive Cancer Center (HCCC) is a nationally recognized tumor center. The HCC (Director: George Weiner MD) is a National Cancer Institute (NCI)-designated cancer center, one of only 60 such centers in the U.S. The Center was recently named a comprehensive cancer center (one of only 37). The HCCC provides in house expertise and nationally ranked affiliated subspecialty care.

Are the departments and faculty of the Optic Nerve Center well known?

The primary subspecialities involved in the Tumors of the Optic Nerve Initiative are ophthalmology, neurology, neurosurgery, cancer therapy, and radiation therapy. The U.S. News and World Report survey identified these specialites as some of "America's Best" including ophthalmology (6th), neurology/neurosurgey (9th), and cancer (38th).

What are the advantages of coming to the Center for optic pathway gliomas?

Optic pathway gliomas are uncommon and consequently few centers have a large experience. The development and study of a large prospective tumor registry (collection of patients over time) would definitely enhance care and advance the field. There is no universally accepted staging system for visual and hypothalamic gliomas. Prospective natural history data might improve our ability to predict the behavior of tumors based upon location or other factors (e.g. association with neurofibromatosis I).

Many of the recent advances in survival with higher quality of life and decreased morbidity (including visual loss) have been made due to large clinical trials. Due to the paucity of cases at any one center, a regional tumor center would be able to recruit and analyze larger numbers of patients for a clinical trial. A multidisciplinary approach is required for the treatment of these tumors.

Radiation therapy to the growing child's brain has significant side effects and newer modalities to reduce these risks are available at UI Health Care. Chemotherapy has been employed to delay the need for radiation therapy in some of these patients and a pediatric neuro-oncology specialist is required as part of the team. Long-term management of these patients is complex, difficult, and requires a multispecialty approach that can best be optimized under a single organizational structure.

What is the advantage of coming to the Center for an optic nerve meningioma?

The development of a large tumor registry for optic nerve meningiomas would also enhance care for these patients. Surgical resection is reserved for patients with severe proptosis or complete or near complete visual loss. Chemotherapy and hormonal therapy have not proven to be effective. New modalities of radiation therapy are currently the mainstay of therapy but no prospective study with large numbers of patients has been performed to date. Long-term follow up is necessary in these patients due to the benign course of the lesion.


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Last modification date: Fri Jun 28, 2002
URL: http://webeye.ophth.uiowa.edu/TONI/FAQ.htm