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What is craniopharyngioma?

Craniopharyngioma is a type of childhood brain tumor. This tumor develops in the area of the brain called the pituitary gland and can extend to other areas of the brain, which can cause hormone dysfunction or vision problems. This tumor grows from cells that in the developing embryo had helped to form the normal pituitary gland. The pituitary gland secretes various hormones into the bloodstream.

Craniopharyngiomas can vary in size and may have cysts (fluid filled sacs). The cysts can become very large in size, and occasionally extend into both sides of the brain. They also can have calcium deposits within them.

How we care for craniopharyngioma

Children with craniopharygioma are treated through the Brain Tumor Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors. Our brain tumor specialists have extensive expertise in treating all types of neural tumors, including craniopharyngiomas. Our patients receive care from neuro-oncologists, neurosurgeons, neurologists and pediatric subspecialists.

Our areas of research for craniopharyngioma

Clinical trials, or research studies evaluating new treatment approaches, are a major offering at Dana-Farber/Boston Children’s. For many children with brain tumors or other rare or hard-to-treat conditions, clinical trials provide new options.

Dana-Farber/Boston Children’s researchers are working on developing new and better treatment options. It’s possible that your child will be eligible to participate in one of our 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 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 tumors, they may be eligible for a number of experimental therapies available through these groups, or from one of our independent clinical investigators.

Craniopharyngioma | Symptoms & Causes

What are the symptoms of craniopharyngioma?

Brain tumors can cause a variety of symptoms in children based primarily on the location of the tumor, including:

  • 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, excessive drinking and urinating, and delayed or early puberty due to damage to the pituitary gland.
  • Increased brain pressure: As the tumor grows larger, it can cause obstructions that block the outflow of spinal fluid. This leads to a buildup of spinal fluid in the brain and causes increased pressure, leading to headache, nausea and vomiting. These are all symptoms of hydrocephalus, which is caused by this fluid buildup inside the brain.
  • Loss of vision: The tumor can put pressure on the nerves of the eyes, which can lead to vision loss in one or both eyes. Some children are diagnosed when they fail a school vision exam.

Keep in mind that the symptoms of a brain tumor may resemble other more common conditions or medical problems. It is important to consult your child's physician for a diagnosis.

What are the causes of craniopharyngioma?

As a parent, you undoubtedly want to know what may have caused your child’s tumor. 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 developing.

Craniopharyngioma | Diagnosis & Treatments

How are craniopharyngiomas diagnosed?

The first step in treating your child is forming an accurate and complete diagnosis. Craniopharyngioma diagnostic tests include:

  • a physical exam and complete medical history.
  • magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images of the structures within the brain and spine
  • computerized tomography scan (CT or CAT scan), a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images of the body from horizontal and vertical perspectives
  • endocrine and ophthalmic evaluations to check for hormone abnormalities or vision problems

Some craniopharyngiomas are very big at the time of diagnosis, and your child may have evidence of increased pressure on the brain. This can be diagnosed with an MRI. If this is the case, the tumor may have to be surgically removed immediately. If your child does not have any evidence of increased pressure, your child’s physician may order a number of different tests,

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 options.

What are the treatment options for craniopharyngiomas?

Your child's physician will determine a specific course of treatment based on several factors, including your child's age, overall health and medical history, the type, location, and size of the tumor and the extent of the disease.

Craniopharyngiomas treatments include:

  • Neurosurgery: Surgery is almost always the first step in treating a craniopharyngioma. Our doctors attempt to remove as much of the tumor as is safely possible. Even if the tumor is completely removed, the tumor can re-grow.
  • Radiation: High-energy waves from a specialized machine damage or shrink tumors. Focused radiation treatments can be used to treat areas of the tumor that cannot be removed surgically. Radiation is typically given daily over a 6-week period. Our patients are referred for proton beam therapy at the New England Proton Center. Radiation is only given focally, as these tumors do not typically spread through the brain and spine.
  • Patients who have their pituitary gland removed due to the diagnosis of craniopharyngioma will require hormone replacement. This means that the removal of the tumor invariably results in a full and complete pituitary hormonal deficit. These children will require life-long replacement with hormones that will be provided under the care of an endocrinologist.

What is the long-term outlook for children with craniopharyngiomas?

The prognosis for children with craniopharyngioma is generally good, with an 80 to 90 percent chance of a cure. Recurrences can occur up to two years after surgery. Therefore, specialized ongoing follow-up care is extremely important.

Children treated for a craniopharyngioma can have difficulties with excess weight gain and sleep and should visit a survivorship clinic to manage disease complications, screen for recurrence and manage late treatment side effects. A typical follow-up visit is likely to include a physical exam, laboratory testing and imaging scans. We will follow your child yearly until 10 years after surgery and then every two years indefinitely.

Through our brain tumor survivorship clinic, the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic, children are able to meet with their neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit. Our multidisciplinary approach and depth of expertise will give your child on-site access to endocrinologists, neuro-psychologists and alternative/complementary therapy specialists. School liaison and psychosocial personnel from the pediatric brain tumor team are also available. In addition, children needing rehabilitation may meet with speech, physical, and occupational therapists during and after their visit.

Craniopharyngioma | Programs & Services