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
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
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:
Orbit and oculoplastic surgery
Adult and pediatric neuro-oncology
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
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
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)
Optical coherence tomography (OCT)
24 hour blood pressure measurements
Video fluorescein angiography
Retinal nerve fiber analysis
What is the Holden Comprehensive Cancer Center?
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
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
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.