Description

Craniopharyngioma is a rare type of non-cancerous tumor in the brain.

Craniopharyngioma begins as a growth of close to the brain cells of the pituitary gland. The pituitary gland produces hormones that control many functions of the body. As a craniopharyngioma grows slowly, it can affect the pituitary gland and nearby structures in the brain.

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

Symptoms

The signs and symptoms of craniopharyngioma may include:

  • Headaches.
  • Changes in vision.
  • Nausea and vomiting.
  • Increased urination.
  • The drowsiness.
  • Memory problems.
  • The loss of balance.
  • Difficulty walking.
  • Changes in personality or behavior.
  • The weight gain and growth retardation in children.

When to see a doctor

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

Causes

It is not clear what causes the craniopharyngioma. Craniopharyngioma begins as a growth of close to the brain cells of the pituitary gland. The pituitary gland produces hormones that control many functions of the body.

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

Risk factors

Health professionals have not found many risk factors for craniopharyngioma. This tumor can occur at any age. But it is more common in children and older adults.

Diagnosis

The diagnosis of craniopharyngioma usually begins with a summary of your medical history and an examination of the symptoms. The evidence for diagnosis of a craniopharyngioma include:

  • Neurological examination. During this test, a health care professional tests of vision, hearing, balance, coordination, reflexes, and growth and development. This can help to show what part of the brain could be affected by the tumor.
  • Blood tests. Blood tests may reveal changes in hormone levels that show is a tumor that affects the pituitary gland.
  • Imaging tests. Imaging tests, capture images of the brain. The pictures can show the size and location of the tumor. Imaging tests include X-rays, ct scans, and Mris. In certain situations, other tests that may be necessary.

Treatment

Craniopharyngioma treatment often starts with the surgery. When possible, surgeons remove the entire tumor. Sometimes the craniopharyngioma may not be completely eliminated. Surgeons may remove the greatest amount of craniopharyngioma as it is safe.

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

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

Surgery

The majority of people with craniopharyngioma undergo a surgery to remove all or the greater part of the tumor. What type of surgery you have depends on the location and size of the tumor.

  • Open craniopharyngioma surgery. It is also called a craniotomy, this operation consists in opening the skull to access the tumor.
  • Minimally invasive craniopharyngioma surgery. It is also called a transsphenoidal procedure, this operation uses special surgical instruments are inserted through the nose. The tools go through a natural step to reach 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 nearby structures. This means that surgeons can't remove the entire tumor. To ensure a good quality of life after the operation, the surgeons remove as much of the tumor as possible. Other treatments may be used after surgery.

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

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

Radiation therapy

Radiation therapy uses powerful energy beams for the control of 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:

  • The 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 health care team carefully the shape and direct the beam of radiation. This helps to provide treatment for the tumor cells and reduces the likelihood of damage to the healthy tissue. These technologies include proton therapy and intensity-modulated radiation therapy, also called intensity-modulated radiation therapy.
  • Stereotactic radiosurgery. Stereotactic radiosurgery is an intense form of radiation treatment. Is the goal of many of the beams of radiation from many angles in the tumor. The 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.

The 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 health care team carefully the shape and direct the beam of radiation. This helps to provide treatment for the tumor cells and reduces the likelihood of damage to the healthy tissue. These technologies include proton therapy and intensity-modulated radiation therapy, also called intensity-modulated radiation therapy.

Chemotherapy

Chemotherapy uses drugs to destroy tumor cells. Chemotherapy can be injected directly into the tumor for the treatment to reach target cells. This makes it less likely to damage the surrounding healthy tissue.

Treatment for papillary craniopharyngioma

The targeted therapy drugs may be a treatment option for a type of craniopharyngioma called papillary craniopharyngioma. This type is not common. In adults, about one out of every three of craniopharyngiomas is the papillary type.

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

Almost all papillary craniopharyngioma cells contain a change in your DNA, called the BRAF gene. Targeted therapy for this change may be a treatment option. Lab tests can show if your craniopharyngioma contains papillary cells. The tests can also tell whether these cells have the BRAF gene change.

Clinical trials

Clinical trials are studies of new treatments. These studies provide an opportunity to test the latest treatments. The risk of side effects may not be known. Ask your health care team if you might be able to be in a clinical trial.

Preparing for your appointment

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

If your healthcare provider thinks 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 is a good idea to be prepared. Here's some information to help you prepare.

What you can do

  • Be conscious of any thing you need to do ahead of time. At the time you make the appointment, be sure to ask if there is anything that you need to do in advance, such as restrict your diet.
  • Write down any symptoms you have, including those that do not seem to be related to the reason for which you scheduled the appointment.
  • Write important personal information, including major stresses or recent life changes.
  • Make a list of all medications, vitamins or supplements you are taking and the dose.
  • Have a family member or friend. Sometimes it can be very difficult to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
  • Write questions to ask their health care team.

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

  • I have a craniopharyngioma?
  • More evidence is needed?
  • What are the treatment options?
  • How much does it cost each treatment to increase my chances of a cure or to prolong my life?
  • What are the possible side effects of each treatment?
  • How will each treatment affect my daily life?
  • There is a treatment option which you think is the best?
  • What would you recommend to a friend or family member in my situation?
  • You should see a specialist?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?
  • What is going to determine whether I should plan for a follow-up visit?

Do not hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

  • When did your symptoms begin?
  • The symptoms been continuous or occasional?
  • How severe are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Craniopharyngioma