Craniopharyngioma
Causes
Brain tumors that begin in the brain
Primary brain tumors originate in the brain itself or in tissues close to it, such as in the brain-covering membranes (meninges), cranial nerves, pituitary gland or pineal gland.
Primary brain tumors begin when normal cells acquire errors (mutations) in their DNA. These mutations allow cells to grow and divide at increased rates and to continue living when healthy cells would die. The result is a mass of abnormal cells, which forms a tumor.
Primary brain tumors are much less common than are secondary brain tumors, in which cancer begins elsewhere and spreads to the brain.
Many different types of primary brain tumors exist. Each gets its name from the type of cells involved. Examples include:
- Gliomas. These tumors begin in the brain or spinal cord and include astrocytomas, ependymoma, glioblastomas, oligoastrocytomas and oligodendrogliomas.
- Meningiomas. A meningioma is a tumor that arises from the membranes that surround your brain and spinal cord (meninges). Most meningiomas are noncancerous.
- Acoustic neuromas (schwannomas). These are benign tumors that develop on the nerves that control balance and hearing leading from your inner ear to your brain.
- Pituitary adenomas. These are mostly benign tumors that develop in the pituitary gland at the base of the brain. These tumors can affect the pituitary hormones with effects throughout the body.
- Medulloblastomas. These are the most common cancerous brain tumors in children. A medulloblastoma starts in the lower back part of the brain and tends to spread through the spinal fluid. These tumors are less common in adults, but they do occur.
- PNETs. Primitive neuroectodermal tumors (PNETs) are rare, cancerous tumors that start in embryonic (fetal) cells in the brain. They can occur anywhere in the brain.
- Germ cell tumors. Germ cell tumors may develop during childhood where the testicles or ovaries will form. But sometimes germ cell tumors move to other parts of the body, such as the brain.
- Craniopharyngiomas. These rare, noncancerous tumors start near the brain's pituitary gland, which secretes hormones that control many body functions. As the craniopharyngioma slowly grows, it can affect the pituitary gland and other structures near the brain.
Cancer that begins elsewhere and spreads to the brain
Secondary (metastatic) brain tumors are tumors that result from cancer that starts elsewhere in your body and then spreads (metastasizes) to your brain.
Secondary brain tumors most often occur in people who have a history of cancer. But in rare cases, a metastatic brain tumor may be the first sign of cancer that began elsewhere in your body.
Secondary brain tumors are far more common than are primary brain tumors.
Any cancer can spread to the brain, but the most common types include:
- Breast cancer
- Colon cancer
- Kidney cancer
- Lung cancer
- Melanoma
Symptoms
The signs and symptoms of a brain tumor vary greatly and depend on the brain tumor's size, location and rate of growth.
General signs and symptoms caused by brain tumors may include:
- New onset or change in pattern of headaches
- Headaches that gradually become more frequent and more severe
- Unexplained nausea or vomiting
- Vision problems, such as blurred vision, double vision or loss of peripheral vision
- Gradual loss of sensation or movement in an arm or a leg
- Difficulty with balance
- Speech difficulties
- Confusion in everyday matters
- Personality or behavior changes
- Seizures, especially in someone who doesn't have a history of seizures
- Hearing problems
When to see a doctor
Make an appointment with your doctor if you have persistent signs and symptoms that concern you.
Brain tumors that begin in the brain
Primary brain tumors originate in the brain itself or in tissues close to it, such as in the brain-covering membranes (meninges), cranial nerves, pituitary gland or pineal gland.
Primary brain tumors begin when normal cells acquire errors (mutations) in their DNA. These mutations allow cells to grow and divide at increased rates and to continue living when healthy cells would die. The result is a mass of abnormal cells, which forms a tumor.
In adults, primary brain tumors are much less common than are secondary brain tumors, in which cancer begins elsewhere and spreads to the brain.
Many different types of primary brain tumors exist. Each gets its name from the type of cells involved. Examples include:
- Gliomas. These tumors begin in the brain or spinal cord and include astrocytomas, ependymomas, glioblastomas, oligoastrocytomas and oligodendrogliomas.
- Meningiomas. A meningioma is a tumor that arises from the membranes that surround your brain and spinal cord (meninges). Most meningiomas are noncancerous.
- Acoustic neuromas (schwannomas). These are benign tumors that develop on the nerves that control balance and hearing leading from your inner ear to your brain.
- Pituitary adenomas. These are mostly benign tumors that develop in the pituitary gland at the base of the brain. These tumors can affect the pituitary hormones with effects throughout the body.
- Medulloblastomas. These are the most common cancerous brain tumors in children. A medulloblastoma starts in the lower back part of the brain and tends to spread through the spinal fluid. These tumors are less common in adults, but they do occur.
- Germ cell tumors. Germ cell tumors may develop during childhood where the testicles or ovaries will form. But sometimes germ cell tumors affect other parts of the body, such as the brain.
- Craniopharyngiomas. These rare, noncancerous tumors start near the brain's pituitary gland, which secretes hormones that control many body functions. As the craniopharyngioma slowly grows, it can affect the pituitary gland and other structures near the brain.
Cancer that begins elsewhere and spreads to the brain
Secondary (metastatic) brain tumors are tumors that result from cancer that starts elsewhere in your body and then spreads (metastasizes) to your brain.
Secondary brain tumors most often occur in people who have a history of cancer. But in rare cases, a metastatic brain tumor may be the first sign of cancer that began elsewhere in your body.
In adults, secondary brain tumors are far more common than are primary brain tumors.
Any cancer can spread to the brain, but common types include:
- Breast cancer
- Colon cancer
- Kidney cancer
- Lung cancer
- Melanoma
Risk factors
In most people with primary brain tumors, the cause of the tumor is not clear. But doctors have identified some factors that may increase your risk of a brain tumor.
Risk factors include:
- Exposure to radiation. People who have been exposed to a type of radiation called ionizing radiation have an increased risk of brain tumor. Examples of ionizing radiation include radiation therapy used to treat cancer and radiation exposure caused by atomic bombs.
- Family history of brain tumors. A small portion of brain tumors occurs in people with a family history of brain tumors or a family history of genetic syndromes that increase the risk of brain tumors.
Alternative medicine
Little research has been done on complementary and alternative brain tumor treatments. No alternative treatments have been proved to cure brain tumors. However, complementary treatments may help you cope with the stress of a brain tumor diagnosis.
Some complementary treatments that may help you cope include:
- Acupuncture
- Art therapy
- Exercise
- Meditation
- Music therapy
- Relaxation exercises
Talk with your doctor about your options.
Coping and support
A diagnosis of a brain tumor can be overwhelming and frightening. It can make you feel like you have little control over your health. But you can take steps to cope with the shock and grief that may come after your diagnosis. Consider trying to:
- Learn enough about brain tumors to make decisions about your care. Ask your doctor about your specific type of brain tumor, including your treatment options and, if you like, your prognosis. As you learn more about brain tumors, you may become more confident in making treatment decisions.
- Keep friends and family close. Keeping your close relationships strong will help you deal with your brain tumor. Friends and family can provide the practical support you'll need, such as helping take care of your house if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by cancer.
- Find someone to talk with. Find a good listener who is willing to listen to you talk about your hopes and fears. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.
Ask your doctor about support groups in your area. Or check your phone book, library or a cancer organization, such as the National Cancer Institute or the American Cancer Society.
Preparing for an appointment
Make an appointment with your doctor if you have any signs or symptoms that worry you. If you're diagnosed with a brain tumor, you may be referred to specialists, such as:
- Doctors who specialize in brain disorders (neurologists)
- Doctors who treat cancer (oncologists)
- Doctors who use radiation to treat cancer (radiation oncologists)
- Doctors who specialize in nervous system cancers (neuro-oncologists)
- Surgeons who operate on the brain and nervous system (neurosurgeons)
- Rehabilitation specialists
It's a good idea to be well-prepared for your appointment. Here's some information to help you get ready, and what to expect from your doctor.
What you can do
- Be aware of any pre-appointment restrictions. 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 any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
- Write down key personal information, including any major stresses or recent life changes.
- Make a list of all medications, vitamins or supplements that you're taking.
- Consider taking a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
- Write down questions to ask your doctor.
Your time with your doctor 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 a brain tumor, some basic questions to ask your doctor include:
- What type of brain tumor do I have?
- Where is my brain tumor located?
- How large is my brain tumor?
- How aggressive is my brain tumor?
- Is my brain tumor cancerous?
- Will I need additional tests?
- What are my treatment options?
- Can any treatments cure my brain tumor?
- What are the benefits and risks of each treatment?
- Is there one treatment you feel is best for me?
- Should I see a specialist? What will that cost, and will my insurance cover it?
- Are there 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?
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions that occur to you.
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may allow time later to cover other points you want to address. Your doctor may ask:
- When did you first begin experiencing symptoms?
- 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?
Overview
Craniopharyngioma is a rare type of noncancerous (benign) brain tumor.
Craniopharyngioma begins near the brain's pituitary gland, which secretes hormones that control many body functions. As a craniopharyngioma slowly grows, it can affect the function of the pituitary gland and other nearby structures in the brain.
Craniopharyngioma can occur at any age, but it occurs most often in children and older adults. Symptoms include gradual changes in vision, fatigue, excessive urination and headaches. Children with craniopharyngioma may grow slowly and may be smaller than expected.
Diagnosis
Tests and procedures used to diagnose craniopharyngioma include:
- Physical exam. Diagnosing a craniopharyngioma usually starts with a medical history review and a neurological exam by your doctor. During this procedure, your vision, hearing, balance, coordination, reflexes, and growth and development are tested.
- Blood tests. Blood tests may reveal changes in hormone levels that indicate a tumor is affecting your pituitary gland.
- Imaging tests. Tests to create images of your brain may include X-rays, magnetic resonance imaging (MRI) and computerized tomography (CT).
Treatment
Craniopharyngioma treatment options include:
Surgery. Surgery to remove all or most of the tumor is most often recommended for people with craniopharyngioma. What type of operation is performed depends on the location and size of your tumor.
Open craniopharyngioma surgery (craniotomy) involves opening the skull to gain access to the tumor. During minimally invasive craniopharyngioma surgery (transsphenoidal procedure), special surgical tools are inserted through your nose. The tools pass through a natural corridor to the tumor, without affecting the brain.
When possible, surgeons remove the entire tumor. But because there are often many delicate and important structures nearby, doctors sometimes don't remove the entire tumor in order to ensure a good quality of life after the operation. In those situations, other treatments may be used after surgery.
Radiation therapy. External beam radiation therapy may be used after surgery to treat craniopharyngioma. This treatment uses powerful energy beams, such as X-rays and protons, to kill tumor cells. During external beam radiation therapy, you lie on a table while a machine precisely aims the energy to the tumor cells.
Specialized external beam radiation technology, such as proton beam therapy and intensity-modulated radiation therapy (IMRT), allows doctors to carefully shape and aim the radiation beam so that it delivers treatment to the tumor cells and spares nearby healthy tissue.
A type of radiation therapy called stereotactic radiosurgery may be recommended in uncommon situations when the tumor doesn't touch the bundle of nerve fibers that transmits visual information from your eye to your brain (optic nerve). Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation on precise points to kill the tumor cells.
Another type of radiation therapy called brachytherapy involves placement of radioactive material directly into the tumor where it can radiate the tumor from the inside.
- Chemotherapy. Chemotherapy is a drug treatment that uses chemicals to kill tumor cells. Chemotherapy can be injected directly into the tumor so that the treatment reaches the target cells and isn't likely to damage nearby healthy tissue.
Treatment for papillary craniopharyngioma. An uncommon type of craniopharyngioma called papillary craniopharyngioma may respond to targeted therapy. Targeted therapy is a drug treatment that focuses on specific abnormalities within the tumor cells that allows them to survive.
Nearly all papillary craniopharyngioma cells contain a mutation in a gene called the BRAF gene. Targeted therapy aimed at this mutation may be a treatment option. Specialized laboratory testing can reveal whether your craniopharyngioma contains papillary cells and whether those cells have the BRAF gene mutation.
Clinical trials. Clinical trials are studies of new treatments or new ways of using existing treatments. A clinical trial gives you the chance to try the latest treatments, but the side effects may not be known. Ask your doctor whether you're eligible to participate in clinical trials.