Craniopharyngioma

Overview

Craniopharyngioma is a rare type of noncancerous brain tumor.

Craniopharyngioma begins as a growth of cells near the brain's pituitary gland. The pituitary gland makes hormones that control many body functions. As a craniopharyngioma slowly grows, it can affect the pituitary gland and other nearby structures in the brain.

Craniopharyngioma can happen at any age, but it occurs most often in children and older adults. Symptoms include changes in vision over time, fatigue, headaches and urinating more often. Children with craniopharyngioma may grow slowly and may be smaller than expected.

Symptoms

Signs and symptoms of craniopharyngioma may include:

• Headaches.
• Vision changes.
• Nausea and vomiting.
• Increased urination.
• Sleepiness.
• Memory troubles.
• Loss of balance.
• Trouble walking.
• Changes in personality or behavior.
• Weight gain and slowed growth in children.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

Causes

It's not clear what causes craniopharyngioma. Craniopharyngioma begins as a growth of cells near the brain's pituitary gland. The pituitary gland makes hormones that control many body functions.

Craniopharyngioma happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In tumor cells, the DNA changes give different instructions. The changes tell the tumor cells to grow and multiply quickly. Tumor cells can keep living when healthy cells would die. This causes too many cells.

Risk factors

Healthcare professionals haven't found many risk factors for craniopharyngioma. This tumor can happen at any age. But it's more common in children and older adults.

Diagnosis

Diagnosing a craniopharyngioma usually starts with a medical history review and a discussion of symptoms. Tests used to diagnose a craniopharyngioma include:

• Neurological exam. During this exam, a healthcare professional tests vision, hearing, balance, coordination, reflexes, and growth and development. This can help show which part of the brain might be affected by the tumor.
• Blood tests. Blood tests may reveal changes in hormone levels that show a tumor is affecting the pituitary gland.
• Imaging tests. Imaging tests capture pictures of the brain. The pictures can show the size and location of the tumor. Imaging tests include X-rays, CTs and MRIs. In certain situations, other tests might be needed.

Treatment

Craniopharyngioma treatment often starts with surgery. When possible, surgeons remove all of the tumor. Sometimes the craniopharyngioma can't be removed completely. Surgeons may remove as much of the craniopharyngioma as is safe.

Radiation therapy may be used after surgery to treat any tumor cells that remain. Using surgery and radiation together often provides good tumor control. This approach also helps lower the risk of complications after surgery.

Other treatments, such as chemotherapy and targeted therapy, might be options in certain situations.

Surgery

Most people with craniopharyngioma have surgery to remove all or most of the tumor. What type of surgery you have depends on the location and size of the tumor.

• Open craniopharyngioma surgery. Also called a craniotomy, this operation involves opening the skull to access the tumor.
• Minimally invasive craniopharyngioma surgery. Also called a transsphenoidal procedure, this operation uses special surgical tools inserted through the nose. The tools go through a natural passage to get to the tumor. This approach minimizes the effect on the brain.

When possible, surgeons remove the entire tumor. However, there are often many delicate and important structures nearby. This means that surgeons sometimes can't remove the entire tumor. To ensure a good quality of life after the operation, surgeons remove as much of the tumor as possible. Other treatments may be used after surgery.

Surgeons do the operation in a way that avoids hurting nearby structures during the operation. Surgeons take care to reduce the risk of vision problems. They work to minimize damage to the hypothalamus. The hypothalamus helps with many functions, including controlling appetite and alertness.

Surgery is sometimes used to relieve a fluid backup on the brain, called hydrocephalus. A tube can be placed to drain the fluid. Often the tube is temporary. Sometimes a permanent tube is needed. A permanent tube, called a shunt, can drain the brain fluid to the abdomen.

Radiation therapy

Radiation therapy uses powerful energy beams to control tumor cells. The energy can come from X-rays, protons or other sources.

Radiation therapy is often used after surgery to treat any tumor cells that are left.

Types of radiation therapy for craniopharyngioma include:

• External beam radiation therapy. During external beam radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

  Specialized external beam radiation technology allows your healthcare team to carefully shape and aim the radiation beam. This helps to deliver treatment to the tumor cells and reduces the chances of hurting healthy tissue. These technologies include proton beam therapy and intensity-modulated radiation therapy, also called IMRT.

• Stereotactic radiosurgery. Stereotactic radiosurgery is an intense form of radiation treatment. It aims many beams of radiation from many angles at the tumor. Stereotactic radiosurgery treatment is typically completed in one or a few treatments.
• Brachytherapy. Brachytherapy involves placing radioactive material directly into the tumor where it can radiate the tumor from the inside.

Chemotherapy

Chemotherapy uses medicines to kill tumor cells. Chemotherapy can be injected directly into the tumor so that the treatment reaches the target cells. This makes it less likely to damage nearby healthy tissue.

Treatment for papillary craniopharyngioma

Targeted therapy medicines might be a treatment option for one type of craniopharyngioma called papillary craniopharyngioma. This type isn't common. In adults, about one out of every three craniopharyngiomas is the papillary type.

Targeted therapy uses medicines that attack specific chemicals in the tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die.

Nearly all papillary craniopharyngioma cells contain a change in their DNA called the BRAF gene. Targeted therapy aimed at this change may be a treatment option. Lab testing can show whether your craniopharyngioma contains papillary cells. Tests also can tell whether those cells have the BRAF gene change.

Clinical trials

Clinical trials are studies of new treatments. These studies provide a chance to try the latest treatments. The risk of side effects might not be known. Ask your healthcare team if you might be able to be in a clinical trial.

Preparing for an appointment

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

If your healthcare professional thinks that you might have a craniopharyngioma, you may be referred to a doctor who specializes in diseases of the nervous system, called a neurologist.

Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins or supplements you're taking and the doses.
• Take a family member or friend along. Sometimes it can be very hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For craniopharyngioma, some basic questions to ask include:

• Do I have a craniopharyngioma?
• Will I need more tests?
• What are the treatment options?
• How much does each treatment increase my chances of a cure or prolong my life?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should I see a specialist?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?