Craniopharyngioma | Diagnosis & Treatment

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.