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Optic glioma
Glioma - optic; Optic nerve glioma; Juvenile pilocytic astrocytoma; Brain cancer - optic glioma
Gliomas are tumors that grow in various parts of the brain. Optic gliomas can affect:
- One or both of the optic nerves that carry visual information to the brain from each eye
- The optic chiasm, the area where the optic nerves cross each other in front of the hypothalamus of the brain
An optic glioma may also grow along with a hypothalamic glioma .
Hypothalamic glioma
A hypothalamic tumor is an abnormal growth in the hypothalamus gland, which is located in the brain.
Causes
Optic gliomas are rare. The cause of optic gliomas is unknown. Most optic gliomas are slow-growing and noncancerous ( benign ) and occur in children, almost always before age 20.
Benign
"Benign" refers to a condition, tumor, or growth that is not cancerous. This means that it does not spread to other parts of the body. It does not ...
There is a strong association between optic glioma and neurofibromatosis Type 1 ( NF1 ).
NF1
Neurofibromatosis-1 (NF1) is an inherited disorder in which nerve tissue tumors (neurofibromas) form in the: Bottom layer of skin (subcutaneous tissu...
Symptoms
The symptoms are due to the tumor growing and pressing on the optic nerve and nearby structures. Symptoms may include:
- Involuntary eyeball movement
- Outward bulging of one or both eyes
- Squinting
-
Vision loss in one or both eyes that starts with the loss of
peripheral
vision and eventually leads to
blindness
Peripheral
Peripheral means "away from the center. " It refers to areas away from the center of the body or a body part. For example, the hands are peripheral ...
ImageRead Article Now Book Mark ArticleBlindness
Blindness is a lack of vision. It may also refer to a loss of vision that cannot be corrected with glasses or contact lenses. Partial blindness mean...
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The child may show symptoms of diencephalic syndrome, which includes:
- Daytime sleeping
- Decreased memory and brain function
- Delayed growth
- Loss of appetite and body fat
Exams and Tests
A brain and nervous system (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 damage to the optic disc.
The tumor may extend into deeper parts of the brain. There may be signs of increased pressure in the brain (intracranial pressure). There may be signs of neurofibromatosis type 1 (NF1).
Neurofibromatosis type 1
Neurofibromatosis-1 (NF1) is an inherited disorder in which nerve tissue tumors (neurofibromas) form in the: Bottom layer of skin (subcutaneous tissu...
The following tests may be performed:
-
Cerebral angiography
Cerebral angiography
Cerebral angiography is a procedure that uses a special dye (contrast material) and x-rays to see how blood flows through the brain.
ImageRead Article Now Book Mark Article -
Examination of tissue removed from the tumor during surgery or
CT
scan-guided
biopsy
to confirm the tumor type
CT
A computed tomography (CT) scan is an imaging method that uses x-rays to create pictures of cross-sections of the body. Related tests include:Abdomin...
ImageRead Article Now Book Mark ArticleBiopsy
A biopsy is the removal of a small piece of tissue for laboratory examination.
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Head CT scan
or
MRI of the head
Head CT scan
A head computed tomography (CT) scan uses many x-rays to create pictures of the head, including the skull, brain, eye sockets, and sinuses.
ImageRead Article Now Book Mark ArticleMRI of the head
A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...
ImageRead Article Now Book Mark Article - Visual field tests during surgery or CT-guided biopsy
Treatment
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.
Surgery to remove the tumor may cure some optic gliomas. Partial removal to reduce the size of the tumor can be done in many cases. This will keep the tumor from damaging normal brain tissue around it. Chemotherapy may be used in some children. Chemotherapy may be especially useful when the tumor extends into the hypothalamus or if vision has been worsened by the tumor's growth.
Radiation therapy may be recommended in some cases when the tumor is growing despite chemotherapy, and surgery is not possible. In some cases, radiation therapy may be delayed because the tumor is slow growing. Children with NF1 usually won't receive radiation due to the side effects.
Corticosteroids may be prescribed to reduce swelling and inflammation during radiation therapy, or if symptoms return.
Support Groups
Organizations that provide support and additional information include:
- Children's Brain Tumor Foundation www.cbtf.org
- Children's Oncology Group www.childrensoncologygroup.org
- Neurofibromatosis Network www.nfnetwork.org
Outlook (Prognosis)
The outlook is very different for each person. Early treatment improves the chance of a good outcome. Careful follow-up with a care team experienced with this type of tumor is important.
Once vision is lost from the growth of an optic tumor, it may not return.
Normally, the growth of the tumor is very slow, and the condition remains stable for long periods. However, the tumor can continue to grow, so it must be monitored closely.
When to Contact a Medical Professional
Call your health care provider for any vision loss, painless bulging of the eye, or other symptoms of this condition.
Prevention
Genetic counseling may be advised for people with NF1. Regular eye exams may allow early diagnosis of these tumors before they cause symptoms.
References
Karcioglu ZA, Haik BG. Eye, orbit, and adnexal structures. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff's Clinical Oncology . 5th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 67.
National Cancer Institute: PDQ childhood astrocytomas treatment. Bethesda, MD: National Cancer Institute. Last modified September 29, 2015. www.cancer.gov/types/brain/hp/child-astrocytoma-treament-pdq . Accessed January 19, 2016.
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology (NCCN guidelines): Central nervous system cancers. Version 1.2015. www.nccn.org/professionals/physician_gls/pdf/cns.pdf . Accessed January 19, 2015.
Sahani M. Neurocutaneous syndromes. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2015:chap 596.
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Neurofibromatosis I, enlarged optic foramen - illustration
This x-ray shows the skull of a child with neurofibromatosis (NF-1). This child developed visual difficulties and was discovered to have a glioma (nerve tumor) in the optic nerve. The tumor has enlarged the bony opening (optic foramen), through which the optic nerve passes. This can be seen on the right side of picture.
Neurofibromatosis I, enlarged optic foramen
illustration
-
Neurofibromatosis I, enlarged optic foramen - illustration
This x-ray shows the skull of a child with neurofibromatosis (NF-1). This child developed visual difficulties and was discovered to have a glioma (nerve tumor) in the optic nerve. The tumor has enlarged the bony opening (optic foramen), through which the optic nerve passes. This can be seen on the right side of picture.
Neurofibromatosis I, enlarged optic foramen
illustration
Review Date: 12/23/2015
Reviewed By: Adam S. Levy, MD, Division of Pediatric Hematology/Oncology, The Children's Hospital at Montefiore, Bronx, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
