Craniopharyngioma

What Are the Signs & Symptoms of a Craniopharyngioma?

When a craniopharyngioma grows and presses on the areas around it, a child might:

• have headaches
• have trouble seeing things to the far left and far right
• have poor balance or trouble walking
• have nausea and vomiting

The child also may:

• need to drink more than usual
  
• need to pee more often than usual
• not go through the usual changes of puberty
• grow slowly or not grow
• not start having periods (menstruation)
• have low blood pressure
• be more tired than usual or sleep a lot
• act differently (have a personality change)

What Causes Craniopharyngioma?

Experts think that craniopharyngiomas grow from leftover pieces of tissue that didn't disappear as expected in early pregnancy when the baby's head, face, and brain are forming.

Who Gets Craniopharyngioma?

Most craniopharyngiomas happen in kids 5 to 14 years old. But they can affect people of any age.

How Are Craniopharyngiomas Diagnosed?

Usually, parents notice one or more of the symptoms of a craniopharyngioma and take their child to a doctor. Other things can cause similar symptoms, so the doctor will:

• ask questions about the symptoms and other medical problems (medical history)
• do an exam
• order blood tests to measure mineral and hormone levels
• do imaging tests, including CAT scan or MRI

Other tests done can include:

• a lumbar puncture (spinal tap)
• a biopsy to test a sample of the tissue. This can involve:
  • surgery through an opening in the skull
  • minimally invasive techniques through the nose or through a needle inserted through a small hole in the skull

How Is Craniopharyngioma Treated?

A team of specialists works together to treat a craniopharyngioma. They include experts in:

• brain and spinal cord disorders (neurology)
• surgery and brain surgery (pediatric surgeons and neurosurgeons)
• hormones and mineral balance (endocrinology)
• diagnosing diseases by examining body tissues, fluids, and organs (pathology)
• imaging (radiology)
• image-guided minimally-invasive procedures (interventional radiology)
• radiation treatment, including proton treatment (radiation oncology)
• behavioral health specialists (psychology and psychiatry)
• nutrition (registered dietitian)

Surgeons usually remove the tumor as the first step in treatment. In many cases, surgery is done without any external incisions, using a camera on a thin tube passed into the nose. Rarely, the tumor is removed through a temporary hole cut in the skull.

The surgeon will put a drain tube (shunt) in at the time of surgery if the child has water on the brain (hydrocephalus). If the surgeon can't completely remove the tumor, the child may also need radiation therapy.

Doctors continue looking for new ways to treat craniopharyngiomas. Some patients might be candidates for a clinical trial. If your child has a craniopharyngioma, talk to the doctor about whether this might be a good treatment option.

What Else Should I Know?

After surgery to remove a craniopharyngioma, the pituitary gland may have some permanent injury. A child may have to take medicines to replace some hormones for life.

Radiation therapy can make the pituitary gland stop working several years after treatment. So children who had this treatment should see a hormone specialist (an endocrinologist) every year.

After treatment, doctors will do follow-up MRIs for several years to check if the tumor has come back.