• Craniopharyngioma Overview

    Craniopharyngioma is a brain tumor that commonly affects children. These tumors grow in the area of the pituitary gland and optic nerve and frequently grow up into the base of a child’s brain. They grow from cells that, in the developing embryo, had helped to form the normal pituitary gland, which secretes various hormones into the bloodstream.

    Craniopharyngioma Treatment at Dana-Farber/Boston Children's

    Patients with craniopharyngioma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Brain Tumor Center. Continue reading to learn more about craniopharyngioma or visit the Brain Tumor Center homepage to learn about our expertise with this condition.

    Symptoms & Diagnosis

    Craniopharyngiomas can cause symptoms in one of three ways:

    • Impaired hormone function – as the tumor grows near the pituitary gland and affects hormone production, there can be a fall-off in a child’s normal growth rate due to lack of growth hormone
    • Increased brain pressure – as the tumor grows up into the base of the brain, it can cause obstructions in the chamber through which fluid circulates, leading to headache, nausea and vomiting, all of which are symptoms of hydrocephalus (fluid buildup inside the brain)
    • Loss of vision – the tumor can put pressure on the optic nerve, which can lead to severe vision loss in one or both eyes (many children are diagnosed when they fail a school vision exam)

    When some children with large tumors are initially diagnosed, they may be quite ill, with increased pressure in the brain and not much time for additional diagnostic testing. In this case, surgery must be performed immediately to decrease intracranial pressure.

    If time is available, additional diagnostic tests include:

    • Computerized tomography (CT or CAT) scan so the doctor can determine the extent of calcium buildup in the tumor (which magnetic resonance imaging can’t) and help identify areas of the tumor that may be difficult to remove.
    • MEI scans, to assess the non-calcified parts of the tumor
    • Endocrine and ophthalmic evaluations to check for hormone abnormalities or vision problems

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Neurosurgery is almost always used to removed as much of the tumor as possible. Even if the tumor is completely removed, the tumor could re-grow from small fragments buried deeper into the brain that cannot be seen at the time of the operation.

    Focused radiation treatments (stereotactic radiosurgery, Gamma Knife or most often proton beam) can be used to treat areas of the tumor that cannot be removed surgically.

    There can be side effects related to the tumor itself or its treatment. Knowing what these side effects are can help physicians prepare for and, in some cases, prevent these symptoms from occurring.

    Progressive or Recurrent Disease

    Craniopharyngioma recurrence is as high as 16 percent after total removal of the tumor. Recurrences can occur up to two years after surgery.

    Long-term Outlook

    The prognosis for children with craniopharyngioma is generally good, with an 80 percent to 90 percent chance of a permanent cure if the tumor can be completely removed with surgery. Many children require lifetime follow-up, including ophthalmology, endocrinology, neuropsychology, neurology and neurosurgery consultations.