• Optic Nerve (Pathway) Glioma in Children

    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.

    Optic Nerve Glioma Treatment at Dana-Farber/Boston Children's

    Children with optic nerve glioma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Glioma Program. Continue reading to learn more about optic nerve glioma or visit the Glioma Program homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    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 at diagnosis. They are most common in children who have the genetic condition neurofibromatosis 1. Due to the position of the tumor, it may also affect the child’s neuroendocrine (hormone system).

    The most common symptoms of optic pathway glioma are:

    • Vision problems such as squinting, difficulty reading, or turning the head to see things out of the corner of the eye
    • Proptosis (eyeball protrusion)
    • Hormonal problems, including abnormal growth, weight gain or loss, and endocrine dysfunction (such as having to urinate frequently)

    In addition to a physical examination, doctors diagnose optic pathway gliomas with

    • Computed tomography (CT or CAT) scan
    • Vision testing

    If a child has neurofibromatosis 1, vision is followed closely. If it starts to worsen, a doctor may order a magnetic resonance imaging (MRI) scan to confirm the diagnosis. After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    The most common treatments for optic nerve glioma are:

    • Chemotherapy – to shrink the tumor and stabilize vision
    • Hormone replacement therapy – generally lifelong therapy if the endocrine systems is affected
    • Radiation therapy – if the tumor is resistant to chemotherapy
    • Alternative medicine (acupuncture/acupressure, therapeutic touch, herbal medicine, etc.) to control pain and treatment side effects

    Surgery is usually not preferred for this type of tumor, but can sometimes relieve symptoms and/or improve vision.

    Progressive or Recurrent Disease

    Optic nerve glioma recurrence may take place many years after initial treatment. It 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 if the patient has not previously been treated with this modality. Chemotherapy and radiation therapy are options for patients who have only been treated surgically.

    Long-term Outlook

    The 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 gliomas. Children may suffer a smaller field of vision, which means they do not have peripheral vision. The odds of complete blindness from these tumors, however, are less than 5 percent.