Optic pathway glioma
We understand that you may have a lot of questions when your child is diagnosed with an optic pathway glioma, including:
- What is it?
- What can we do about it?
- How serious is it?
- How will it affect my child long-term?
We’ve provided some answers to these questions here, and when you meet with our experts, we can talk with you more about your child’s diagnosis and treatment. Learn more about our experts in the Brain Tumor Program and what resources are available to you and your family.
What is an optic pathway glioma?
An optic pathway glioma is a slow-growing brain tumor that arises in or around the optic nerve. As the tumor progresses, it presses the optic nerve, causing a child’s vision to worsen.
The optic system is located near the hormone center of the brain, which is essential for the body's endocrine function (such as hormone production, salt and water balance, appetite and sleep centers). This means that an optic pathway glioma may affect these functions.
What complications are associated with optic pathway glioma?
Complications associated with optic pathway glioma include:
Can the damage to my child’s vision be reversed?
It depends. For a while, if the nerve is squeezed and then the squeezing stops, it will regain its former shape and start to work again, like a sponge that has been squeezed. But if the squeezing goes on for longer than around six months, there’s less of a chance that the nerve will recover and there is a high risk for blindness. Overall, blindness only occurs in 5 percent cases of optic pathway glioma since usually it is repaired before the nerve is squeezed.
What is the expected outcome after treatment?
For all types of optic pathway glioma, whether those with or without neurofibromatosis 1, the cure rate is above 90 percent.
Is it life-threatening?
It can be life threatening, but the risk for death is extremely low. Early intervention will not reduce the risk of death, but it will improve the outlook on quality of life. If death does occur, it’s not from the tumor itself, but from the damage on the hypothalamus from the pressure applied by the tumor. A damaged hypothalamus could result in a loss of control of salt levels. High salt levels can cause death.
Can it spread to other parts of the body?
Optic pathway glioma can move throughout different parts of the brain and spine. There is a blood brain barrier that serves to keep infections out, and also ends up keeping tumors in. It is very rare, below 5 percent of the time, for the glioma to move beyond the brain and spine.
Will my child be blind?
While the risk of becoming blind as a result of optic pathway glioma is far less than it used to be, the best predictor of how much vision your child will have is the state of his vision when he was diagnosed. The better his vision at diagnosis, and the better his vision is likely to be after treatment. Additionally, the younger the child is at diagnosis, the odds are less that he’ll be blind. In general blindness very rarely results from optic pathway glioma.
What is treatment usually like?
Treatment can include surgery, chemotherapy radiation and or alternative medicines like acupuncture. Since gliomas often resurface, your child will receive multiple follow up MRI scan visits to see if it has come back.
Where will my child be treated?
Children treated through Dana-Farber/Children’s Hospital Cancer Center receive outpatient care at the Jimmy Fund Clinic, which is located on the third floor of the Dana Farber Cancer Institute. If your child needs to be admitted to the hospital overnight, he’ll stay at Children’s Hospital Boston, on the ninth floor of the Berthiaume building.
Do optic pathway gliomas ever go away on their own?
Yes, for children with neurofibromatosis 1. About two-thirds of those children experience spontaneous remissions of their optic pathway gliomas. It’s very uncommon for children who don’t have NF1 to go into remission without treatment.
What causes optic pathway glioma?
Aside from the genetic disorder neurofibromatosis 1, there is no known cause. Optic pathway glioma occurs in 15 percent of patients with neurofibromatosis 1.
Signs and symptoms
What are the symptoms of an optic pathway glioma?
Optic pathway gliomas are usually diagnosed within the first two years of life. While each child is different, common symptoms of an optic pathway glioma include:
1. Problems with vision
If a child doesn’t have NF1, and isn’t having his vision monitored routinely, a parent or teacher may notice that he’s:
- squinting a lot
- talking aloud about objects he sees to try to make sense of what he’s seeing since he’s having trouble seeing them
- having trouble reading
- turning his head to be able to see things out of the corner of his eye
If a child is having trouble seeing, an ophthalmologist can tell whether the problem is with the nerve or with the lens—there are lots of kids that need glasses and it has nothing to do with having an optic glioma.
2. Eyeball protrusion (proptosis)
With proptosis, the tumor has grown to the extent that it’s pushing the eye outward from its socket. Usually, an eye with proptosis has very little vision.
3. Hormonal problems
Because the optic pathway system lies in close proximity to the hormone center of the brain, your child may display:
- abnormal growth
- weight gain
- weight loss
- endocrine dysfunction (such as having to urinate frequently)
Q: What’s the long-term outlook for a child with an optic pathway glioma?
A: The overwhelming majority of children survive their optic pathway glioma. Being older at diagnosis and having neurofibromatosis 1 are correlated with better outcomes.
Q: How will this brain tumor affect my child’s mental abilities?
A: These brain tumors do not directly affect a child’s intelligence, however, since much of learning is through seeing, such as reading a book, a child may have challenges learning in the classroom. We will work with your child’s teachers to make sure the classroom can accommodate his learning needs. For example, moving a child’s seat may help the child he cannot see from a certain side.
Q: What services are available to help my child and my family cope?
A:We offer many services to help you, your child and your family get through this difficult time. Read about our support services.
Q: Where will my child be treated?
A:Children treated through Dana-Farber/Children’s Hospital Cancer Center receive outpatient care at the Jimmy Fund Clinic, which is located on the third floor of the Dana Farber Cancer Institute. If your child needs to be admitted to the hospital overnight, he’ll stay at Children’s Hospital Boston, on the ninth floor of the Berthiaume building.
Questions to ask your doctor
After your child is diagnosed with an optic pathway glioma, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you. Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.
Some of the questions you may want to ask include:
- Has my child’s tumor spread?
- How will my child’s tumor be treated?
- How long will my child need to be in the hospital?
- What are the possible short and long-term complications of treatment? How will they be addressed?
- What is the likelihood that my child will be cured?
- What is the likelihood that my child’s vision will be spared?
- What happens if the tumor returns?
What else should I know at this point?
Back to School program
At age 11, Ronald (R.J.) Agostinelli was diagnosed with acute lymphoblastic leukemia, a cancer of the white blood cells. He missed seven months of elementary school while having chemotherapy. R.J. talks about what it was like returning to his class after a long absence.