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Alternative Names Return to topGlioma - optic; Optic nerve glioma
Definition Return to top
Gliomas are tumors that grow in various parts of the brain. Optic gliomas can affect:
An optic glioma may also grow along with a hypothalamic glioma.
Causes Return to top
Optic gliomas are rare. The cause of optic gliomas is unknown. Most optic gliomas are slow-growing and non-cancerous (benign) and occur in children, almost always before age 20.
There is a strong association between optic glioma and neurofibromatosis Type 1 (NF1).
Symptoms Return to top
The symptoms are due to the tumor growing and pressing on the optic nerve and nearby structures. Symptoms may include:
The patient may show symptoms of Diencephalic syndrome, which includes delayed growth, loss of appetite and body fat, daytime sleeping and decreased memory and intellectual function.
Exams and Tests Return to top
A neurologic examination reveals a loss of vision in one or both eyes. There may be changes in the optic nerve, including swelling or scarring of the nerve, or paleness and atrophy of the optic disk.
The tumor may extend into deeper locations of the brain. There may be signs of increased pressure within the brain (intracranial pressure). There may be signs of neurofibromatosis type 1 (NF1).
The following tests may be performed:
Treatment Return to top
Treatment varies with the size of the tumor and the general health of the person. The goal may be to cure the disorder, relieve symptoms, or improve vision and comfort.
Surgical removal may cure some optic gliomas. Partial removal to reduce the bulk of the tumor is feasible in many cases, which will minimize pressure-induced damage from the tumor.
Radiation therapy may be advised in some cases where the tumor is extensive and surgery is not possible. In some cases, radiation therapy may be delayed because of the slow growth that this tumor typically displays.
Corticosteroids may be prescribed to reduce swelling and inflammation during radiation therapy, or if symptoms return.
Chemotherapy could be useful in a number of children. Chemotherapy might be especially useful when the tumor extends into the hypothalamus.
Support Groups Return to top
For organizations that provide support and additional information, see blindness resources.
Outlook (Prognosis) Return to top
The outlook is highly variable. Early treatment improves the chance of a good outcome. Many tumors are curable with surgery, while others return.
Normally, the growth of the tumor is very slow, and the condition remains stable for long periods of time. However, in adults and some childhood cases where the optic chiasm is involved, the tumor behaves aggressively.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if you have any vision loss, painless bulging of your eye forwards, or other symptoms of this condition.
Prevention Return to top
Genetic counseling may be advised for people with neurofibromatosis-1. Regular eye exams may allow early diagnosis of these tumors before they cause symptoms.
References Return to top
Homes LR, Munzenrider JE, Elner VM, Bardenstein DS, Lichter AS. Eye, orbit, and adnexal structures. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Clinical Oncology. 3rd ed. Philadelphia, PA: Churchill Livingstone;2004;chap 70.
Olitsky SE, Hug D, Smith LP. Abnormalities of the optic nerve. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. St. Louis, MO: WB Saunders;2007;chap 608.Update Date: 6/10/2008 Updated by: James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.