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Optic Pathway Glioma

  • "The advantage of having seven dedicated pediatric neurosurgeons who do nothing but gliomas means that we've got exactly the right expertise."

    Mark Kieran, MD, PhD, clinical director, Brain Tumor Center

    If your child has been diagnosed with an optic pathway glioma, you’ll have concerns and questions about his health, treatment, recovery and other issues. It may comfort you to know that these types of brain tumors have a very high survival rate, above 90 percent and that Dana-Farber/Boston Children’s Cancer and Blood Disorders Center is a world leader in treating childhood cancer. We specialize in innovative, family-centered care that supports your child and family every step of the way.

    Optic pathway gliomas are brain tumors that arise in or around the optic nerve (nerve that connects the eye to the brain). They are serious, but have an extremely high cure rate.

    An optic pathway glioma is most common in children who have the genetic condition neurofibromatosis 1 (NF1). In these cases, about two-thirds of the time, the tumor stop growing and disappears forever on its own without treatment.

    • Optic pathway gliomas account for 5 percent of all childhood brain tumors.
    • They may affect one eye (unilateral) or both eyes (bilateral).
    • The majority of people (75 percent) with optic pathway gliomas are younger than 10; most are younger than 5 at the time of diagnosis.
    • Girls and boys are equally affected.
    • There are no racial or ethnic groups that are at more risk than others.
    • Due to the position of the optic nerve, a tumor may also affect your child’s endocrinological (hormone) system.
    • Symptoms of optic pathway glioma may include worsening vision, growth problems or precocious puberty.
    • Treatment options may include chemotherapy, surgery and radiation.
    • Cure rate is above 90 percent.
    • Blindness can occur, but only occurs in 5 percent of cases.

    How Dana-Farber/Children’s Hospital Cancer Center approaches optic pathway gliomas

    The Dana-Farber/Boston Children’s Cancer and Blood Disorders Center is an integrated pediatric oncology program that provides all the services of both a leading cancer center and a pediatric hospital. This team approach ensures that your child's treatment plan is carefully developed and coordinated. We integrate expertise from the following specialists:

    • pediatric oncologists, surgical oncologists and radiation oncologists
    • pediatric experts from every medical subspecialty, such as ophthalmology and radiology
    • highly skilled and experienced pediatric oncology nurses
    • psycho-oncologists, resource and education specialists, financial coordinators, Child Life specialists, pharmacists and many others to help manage your child’s physical, emotional and everyday needs
    • unique programs like Home Visits, an outreach program that helps newly diagnosed families adjust to life after a cancer diagnosis

    We hold a weekly brain tumor clinic for newly diagnosed children currently receiving treatment. Each time you come for an appointment, you meet with every specialist on your child’s team, from your pediatric neuro-oncologist, neurologist and neurosurgeon to your pediatric endocrinologist, psycho-oncologist and school liaison. We also offer access to:

    • high-tech resources like the intra-operative MRI, which allows our pediatric neurosurgeons to visualize the tumor as they operate with MRI scans. This means they can remove as much of the tumor as possible, and sometimes eliminate additional surgeries.
    • expert neuropathological review, using advanced molecular diagnostic testing, to identify your child’s exact type of tumor. This information helps predict which treatments are more likely to work.
    • unique Phase I clinical trials that offer treatment options beyond standard therapy
    • one of the nation’s few dedicated pediatric brain tumor survivorship programs. This weekly clinic offers ongoing care to manage late effects caused by your child’s tumor or the treatment he received.

  • 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 Center 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.

    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.

    Causes

    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)

    FAQ

    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.

    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?
  • The first step in treating your child is forming an accurate and complete diagnosis. Diagnostic procedures may include:

    • examination and visual testing—An ophthalmologist (vision specialist) examines your child and conduct comprehensive testing of his eyesight. This information is used in conjunction with MRI findings to make an accurate diagnosis.
    • computerized tomography scan (also called a CT or CAT scan)—a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays. For optic pathway glioma, a CT scan of the brain is usually done.

    If your child has NF1, we follow his vision very carefully. If it starts to worsen, we’ll perform an MRI scan to confirm that it’s an optic pathway glioma, and then determine the treatment.

    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 treatment options.

  • We understand how difficult a diagnosis of optic pathway glioma can be, both for your child and for your whole family. That's why our physicians are focused on family-centered care: From your first visit, you'll work with a team of professionals who are committed to supporting all of your family's physical and psychosocial needs. We'll work with you to create a care plan that's best for your child.

    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 (network of gland controlling hormones), he may also need hormone replacement therapy, possibly for the rest of his life.

    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:

    • acupuncture/acupressure
    • therapeutic touch
    • massage
    • herbal supplements
    • dietary recommendations

    Talk to your child's physician about whether complementary or alternative medicine might be a good option for your child.

    What is the long-term outlook for a child with an optic pathway glioma?

    As with any cancer, the prognosis can vary greatly from child to child. Luckily, the survival rate is very high, above 90 percent, and prompt medical attention and aggressive therapy usually contribute to your child's best outcome. Your child may suffer from a smaller field of vision, which may mean he does not have peripheral vision, but the odds of complete blindness are below 5 percent.

    How will you follow up with my child?

    After treatment, we generally see children 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.

    What specialized treatment do you offer for my child's vision problems?

    Patients 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.

    What will long-term care for my child be like?

    Lifelong follow-up care is crucial if your child has been treated for optic pathway glioma. It's important for your child to visit a survivorship clinic every year to:

    • manage disease complications
    • screen for recurrence of cancer and/or secondary cancers
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • physical exam
    • laboratory testing
    • imaging scans

    Through the brain tumor survivorship clinic at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.

    Coping and support

    In addition to providing medical care, we strive to also meet all of your family's emotional and quality-of-life needs. We know that unfamiliar places, especially hospitals, often create anxiety and fear for a child. We also know this isn't an easy time or task for parents, who have their own fears and concerns about the situation. Our family support services will help address many of your needs and concerns.


  • The Brain Tumor Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center is involved with several research efforts, including Phase I Children's Oncology Group clinical trials.

    Clinical trials

    For many children with rare or hard-to-treat conditions, clinical trials provide new options.

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