An optic nerve glioma (also called an optic pathway glioma) is a slow-growing brain tumor that arises in or around the optic nerve, which connects the eye to the brain. As the tumor progresses, it presses on the optic nerve, causing a child’s vision to worsen. Blindness can occur, but only in about 5 percent of cases. While these are serious tumors, they have a high cure rate.
Nearly 75 percent of optic nerve gliomas, which may affect one or both eyes, occur in children younger than 10, with most younger than 5 years of age at the time of diagnosis. Optic nerve gliomas account for 5 percent of all childhood brain tumors. Because the optic system is located near the hormone center of the brain, these tumors can affect the body's endocrine functions, such as hormone production, salt and water balance, appetite and sleep.
Children with optic nerve gliomas are treated at Dana-Farber/Boston Children's through our Glioma Program – one of the world’s largest pediatric glioma treatment programs. Our internationally-recognized pediatric brain tumor specialists have extensive expertise in treating all types of gliomas, including optic nerve gliomas. Our glioma specialists – a team of neuro-oncologists, surgeons, pathologists and radiation oncologists – focus solely on the care of children diagnosed with gliomas. Our program also offers families the chance to have their child's tumor molecularly profiled (as long as a biopsy can be taken), which may help identify opportunities for targeted treatment (precision medicine).
nerve gliomas are a type of glioma, slow growing tumors that arise from glial cells in your
child's brain. Glial cells are responsible for supporting and nourishing the
neurons in the brain.
are divided into four grades, depending on the tumor cells' appearance under microscope;
the higher a tumor's grade number, the more severe it is. Grades 1 and 2 are
considered low-grade gliomas and account for about two-thirds of
all pediatric tumors. Grades 3 and 4 are considered high-grade gliomas.
Brain tumors can cause a variety of symptoms
in children based on their age and the location of the tumor. Keep in mind that
the symptoms of an optic nerve glioma may resemble other more common conditions
or medical problems, such as needing glasses to correct poor vision. It is
important to consult your child's physician for a diagnosis.
The most common optic pathway glioma symptoms are:
As a parent, you undoubtedly want to know
what may have caused your child’s tumor. In most cases, the cause of optic
nerve gliomas is not known. It’s important to understand that these and other
brain tumors most often occur with no known cause. There’s nothing that you
could have done or avoided doing that would have prevented the tumor from
Optic pathway gliomas are more common in kids who have a genetic
condition called neurofibromatosis 1. NF1 can
cause tumors to form, and optic pathway gliomas occur in about 15% of children
with this disease. In these cases, about two-thirds of the time, the tumor stops
growing and disappears forever on its own without treatment.
The first step in treating
your child is forming an accurate and complete diagnosis. Optic pathway gliomas
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.
nerve gliomas are typically diagnosed using a combination of imaging and vision
testing. Your child's doctor will likely order tests including:
There may be other diagnostic tests that your
doctor will discuss with you depending on your child's individual situation.
After we complete all necessary tests, our experts meet to review and discuss
what they have learned about your child's condition. Then we will meet with you
and your family to discuss the results and outline the best possible treatment
Your child's physician will determine a specific course of treatment
based on several factors, including:
Most often, optic pathway
glioma is treated with chemotherapy – a drug treatment that works by interfering
with the cancer cell's ability to grow or reproduce – to shrink the tumor and
stabilize or improve vision.
If the tumor has affected
your child's endocrine system (a network of gland controlling hormones), he or
she may also need hormone replacement therapy, possibly for the rest of his or
While surgery is not commonly
performed for this type of brain tumor, it can sometimes relieve symptoms
and/or improve vision. Our expert pediatric neural surgeons have a wealth of
experience in determining whether surgery is the right option.
Most of the time,
chemotherapy can stop the progression of optic pathway gliomas. But if your
child's tumor is resistant to chemotherapy, radiation may also be an option.
Radiation therapy uses high-energy rays (radiation) from a specialized machine
to damage or kill cancer cells and shrink tumors.
Our cancer treatment center
has specialists who deliver complementary or alternative medicines. These
treatments, which may help control pain and side effects of therapy, include:
Talk to your child's physician about whether
complementary or alternative medicine might be a good option for your child.
Scientists at both the Dana-Farber Cancer Institute and Boston Children’s Hospital are
conducting numerous research studies to better clinicians' understanding and
treatment of brain tumors and gliomas. Our research program offers patients
unparalleled access to clinical trials in which children can receive the newest
treatments. We are consistently one of the most well funded pediatric brain
tumor centers in the United States, and we pride ourselves on rapidly bringing
our discoveries to the aid of our patients.
Dana-Farber/Boston Children's houses the Pediatric Low-Grade Astrocytoma (PLGA) Program, the
world's only multidisciplinary clinical and research program dedicated to
pediatric low-grade gliomas. Established in 2007 with support from the PLGA
Foundation, the program takes a multifaceted approach to finding more
effective, less toxic treatments and a cure for children battling brain tumors,
and has become the standard bearer for the research and care of pediatric brain
tumors. Our pediatric neuro-oncologists, including Mark Kieran,
MD, PhD, clinical director of the Brain Tumor Center at
Dana-Farber/Boston Children's, and Pratiti
(Mimi) Bandopadhayay, MBBS, PhD, are actively contributing to these
efforts. Our program has contributed to international
research efforts that have identified genomic drivers that contribute to growth
of pilocytic astrocytoma. Specifically, we have identified genes that are
commonly mutated in pilocytic astrocytoma. These findings are guiding clinical
trials examining the activity of new drugs specifically for children with optic
Clinical trials, or research
studies evaluating new treatment approaches, are a major offering at
Dana-Farber/Boston Children’s. Clinical trials are very important for children
with hard-to-treat or relapsed conditions.
It’s possible that your child
will be eligible to participate in one of the Glioma Program’s current brain tumor clinical trials.
In addition to launching our own clinical trials, we also offer trials
available through collaborative groups such as the Children's Oncology Group
(COG) and the Pacific Pediatric
Neuro-Oncology Consortium (PNOC). We are also the New England Phase I
Center of the Children's Oncology Group. If your child has progressive or
recurrent tumor, she may be eligible for a number of experimental therapies
available through these groups, or from one of our independent clinical
survival rate for optic pathway gliomas is near 90 percent. Older children and
those with neurofibromatosis 1 have better outcomes. In fact, two-thirds of
children with NF1 experience spontaneous remission of their optic pathway
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.
The odds of complete blindness from these tumors is less than
5 percent, but children may experience a smaller field of vision as a result of
the tumor, which means they do not have peripheral vision. The best predictor of how much
vision your child will have is how good his or her vision was when he was
diagnosed. Generally, better original vision correlates with better vision
following treatment. Additionally, children who are relatively young at
diagnosis tend to preserve their vision better than older kids.
In many cases, any damage to your child's vision may be
the nerve is squeezed and the squeezing stops, it will regain its former shape
and start to work again, like a sponge. 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.
Optic nerve glioma
recurrence may take place many years after initial treatment. A glioma usually
recurs in the same place as the original tumor, but can also occur in other
parts of the brain or spinal cord. Local radiation therapy is the usual
treatment. Chemotherapy and radiation therapy are options for patients who have
only been treated surgically.
What kind of long-term care
will my child need for optic nerve glioma?
Because these tumors can
recur or spread, and because cancer survivors face unique life-long health
challenges, we will generally see your child for a follow-up MRI every three months for a
year, then every six months for another year, and then yearly after that.
Throughout, your child will have a vision exam every three months. Follow up
care is crucial and is overseen by our brain tumor survivorship clinic.
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
may benefit from the Low Vision Service at Boston Children's Hospital, to help find the best visual aids for
your child. Additionally there is Vision Therapy Service which may help to improve vision through therapy.
Mark Kieran, MD, PhD, provides a one-hour presentation for parents on latest approaches to treating brain tumors; sponsored by American Brain Tumor Association.