Medulloblastoma Causes Symptoms and treatment

 

Medulloblastoma


Overview

Medulloblastoma (muh-dul-o-blas-TOE-muh) is a cancerous (malignant) brain tumor that starts in the lower back part of the brain, called the cerebellum. The cerebellum is involved in muscle coordination, balance and movement.

See:  Brain tumor

Medulloblastoma tends to spread through cerebrospinal fluid (CSF) — the fluid that surrounds and protects your brain and spinal cord — to other areas around the brain and spinal cord. This tumor rarely spreads to other areas of the body.

Medulloblastoma is a type of embryonal tumor — a tumor that starts in the fetal (embryonic) cells in the brain. Based on different types of gene mutations, there are at least four subtypes of medulloblastoma. Though medulloblastoma is not inherited, syndromes such as Gorlin's syndrome or Turcot's syndrome might increase the risk of medulloblastoma.

Signs and symptoms of medulloblastoma may include headaches, nausea, vomiting, tiredness, dizziness, double vision, poor coordination, unsteady walk and other concerns. These symptoms may be related to the tumor itself or be due to the buildup of pressure within the brain.

Medulloblastoma can occur at any age, but most often occurs in young children. Though medulloblastoma is rare, it's the most common cancerous brain tumor in children. Children need to be seen at a center that has a team of pediatric specialists with expertise and experience in pediatric brain tumors, with access to the latest technology and treatments for children.

Diagnosis

The process of diagnosis usually starts with a medical history review and a discussion of signs and symptoms. Tests and procedures used to diagnose medulloblastoma include:

  • Neurological exam. During this procedure, vision, hearing, balance, coordination and reflexes are tested. This helps determine which part of the brain might be affected by the tumor.
  • Imaging tests. Imaging tests can help determine the location and size of the brain tumor. These tests are also very important to identify pressure or blockage of the CSF pathways. A computerized tomography (CT) scan or magnetic resonance imaging (MRI) may be done right away. These tests are often used to diagnose brain tumors. Advanced techniques, such as perfusion MRI and magnetic resonance spectroscopy, also may be used.
  • Tissue sample testing (biopsy). A biopsy is usually not done, but it may be recommended if the imaging tests are not typical of medulloblastoma. The sample of suspicious tissue is analyzed in a lab to determine the types of cells.
  • Removal of cerebrospinal fluid for testing (lumbar puncture). Also called a spinal tap, this procedure involves inserting a needle between two bones in the lower spine to draw out cerebrospinal fluid from around the spinal cord. The fluid is tested to look for tumor cells or other abnormalities. This test is only done after managing the pressure in the brain or removing the tumor.

Treatment

Treatment for medulloblastoma usually includes surgery followed by radiation or chemotherapy, or both. Age and general health, tumor subtype and location, tumor grade and extent, and other factors play a role in treatment decisions. Options include:

  • Surgery to relieve fluid buildup in the brain. A medulloblastoma may grow to block the flow of cerebrospinal fluid, which can cause a buildup of fluid that puts pressure on the brain (hydrocephalus). Surgery to create a pathway for the fluid to flow out of the brain (external ventricular drain or ventriculoperitoneal shunt) may be recommended. Sometimes this procedure can be combined with surgery to remove the tumor.
  • Surgery to remove the medulloblastoma. A pediatric or adult brain surgeon (neurosurgeon) removes the tumor, taking care not to harm nearby tissue. But sometimes it's not possible to remove the tumor entirely because medulloblastoma forms near critical structures deep within the brain. All patients with medulloblastoma should receive additional treatments after surgery to target any remaining cells.
  • Radiation therapy. A pediatric or adult radiation oncologist administers radiation therapy to the brain and spinal cord using high-energy beams, such as X-rays or protons, to kill cancer cells. Standard radiation therapy can be used, but proton beam therapy — available at a limited number of major health care centers in the United States — delivers higher targeted doses of radiation to brain tumors, minimizing radiation exposure to nearby healthy tissue.
  • Chemotherapy. Chemotherapy uses drugs to kill tumor cells. Typically, children and adults with medulloblastoma receive these drugs as an injection into the vein (intravenous chemotherapy). Chemotherapy may be recommended after surgery or radiation therapy, or in certain cases, at the same time as radiation therapy. In some cases, high dose chemotherapy followed by stem cell rescue (a stem cell transplant using the patient's own stem cells) may be used.
  • Clinical trials. Clinical trials enroll eligible participants to study the effectiveness of new treatments or to study new ways of using existing treatments, such as different combinations or timing of radiation therapy and chemotherapy. These studies provide a chance to try the latest treatment options, though the risk of side effects may not be known. Talk with your doctor for advice.

Diagnosis

If it's suspected that you have a brain tumor, your doctor may recommend a number of tests and procedures, including:

  • A neurological exam. A neurological exam may include, among other things, checking your vision, hearing, balance, coordination, strength and reflexes. Difficulty in one or more areas may provide clues about the part of your brain that could be affected by a brain tumor.
  • Imaging tests. Magnetic resonance imaging (MRI) is commonly used to help diagnose brain tumors. Sometimes a dye is injected through a vein in your arm during your MRI study.

    A number of specialized MRI scan components — including functional MRI, perfusion MRI and magnetic resonance spectroscopy — may help your doctor evaluate the tumor and plan treatment.

    Sometimes other imaging tests are recommended in certain situations, including computerized tomography (CT) and positron emission tomography (PET).

  • Collecting and testing a sample of abnormal tissue (biopsy). A biopsy can be performed as part of an operation to remove the brain tumor, or a biopsy can be performed using a needle.

    A stereotactic needle biopsy may be done for brain tumors in hard to reach areas or very sensitive areas within your brain that might be damaged by a more extensive operation. Your neurosurgeon drills a small hole into your skull. A thin needle is then inserted through the hole. Tissue is removed using the needle, which is frequently guided by CT or MRI scanning.

    The biopsy sample is then viewed under a microscope to determine if it's cancerous or benign. Sophisticated laboratory tests can give your doctor clues about your prognosis and your treatment options. Studying your biopsy sample and determining exactly which type of brain tumor you have is a complex process. If you're uncertain about your diagnosis, consider seeking a second opinion at a medical center where many brain biopsies are evaluated every year.

Treatment

Treatment for a brain tumor depends on the type, size and location of the tumor, as well as your overall health and your preferences.

Surgery

If the brain tumor is located in a place that makes it accessible for an operation, your surgeon will work to remove as much of the brain tumor as can be done safely.

Some brain tumors are small and easy to separate from surrounding brain tissue, which makes complete surgical removal possible. Other brain tumors can't be separated from surrounding tissue or they're located near sensitive areas in your brain, making surgery risky. In these situations, your doctor removes as much of the tumor as is safe.

Even removing a portion of the brain tumor may help reduce your signs and symptoms.

Surgery to remove a brain tumor carries risks, such as infection and bleeding. Other risks may depend on the part of your brain where your tumor is located. For instance, surgery on a tumor near nerves that connect to your eyes may carry a risk of vision loss.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays or protons, to kill tumor cells. Radiation therapy can come from a machine outside your body (external beam radiation), or, very rarely, radiation can be placed inside your body close to your brain tumor (brachytherapy).

External beam radiation can focus just on the area of your brain where the tumor is located, or it can be applied to your entire brain (whole-brain radiation). Whole-brain radiation is most often used to treat cancer that spreads to the brain from some other part of the body and forms multiple tumors in the brain.

Traditionally, radiation therapy uses X-rays, but a newer form of this treatment uses proton beams. Proton beam therapy allows doctors to control the radiation more precisely. It may be helpful for treating brain tumors in children and tumors that are very close to sensitive areas of the brain. Proton beam therapy isn't as widely available as traditional X-ray radiation therapy.

Side effects of radiation therapy depend on the type and dose of radiation you receive. Common side effects during or immediately following radiation include fatigue, headaches, memory loss, scalp irritation and hair loss.

Radiosurgery

Stereotactic radiosurgery is not a form of surgery in the traditional sense. Instead, radiosurgery uses multiple beams of radiation to give a highly focused form of radiation treatment to kill the tumor cells in a very small area. Each beam of radiation isn't particularly powerful, but the point where all the beams meet — at the brain tumor — receives a very large dose of radiation to kill the tumor cells.

There are different types of technology used in radiosurgery to deliver radiation to treat brain tumors, such as a Gamma Knife or linear accelerator.

Radiosurgery is typically done in one treatment, and usually you can go home the same day.

Chemotherapy

Chemotherapy uses drugs to kill tumor cells. Chemotherapy drugs can be taken orally in pill form or injected into a vein (intravenously). The chemotherapy drug used most often to treat brain tumors is temozolomide (Temodar). Other chemotherapy drugs may be recommended depending on the type of cancer.

Chemotherapy side effects depend on the type and dose of drugs you receive. Chemotherapy can cause nausea, vomiting and hair loss.

Tests of your brain tumor cells can determine whether chemotherapy will be helpful for you. The type of brain tumor you have also is helpful in determining whether to recommend chemotherapy.

Targeted drug therapy

Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die.

Targeted therapy drugs are available for certain types of brain tumors, and many more are being studied in clinical trials. Your doctor may have your tumor cells tested to see whether targeted therapy is likely to be an effective treatment for your brain tumor.

Rehabilitation after treatment

Because brain tumors can develop in parts of the brain that control motor skills, speech, vision and thinking, rehabilitation may be a necessary part of recovery. Depending on your needs, your doctor may refer you to:

  • Physical therapy to help you regain lost motor skills or muscle strength
  • Occupational therapy to help you get back to your normal daily activities, including work, after a brain tumor or other illness
  • Speech therapy with specialists in speech difficulties (speech pathologists) to help if you have difficulty speaking
  • Tutoring for school-age children to help kids cope with changes in their memory and thinking after a brain tumor

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 home 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 hear 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 your 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 prepared for your appointment. Here's some information to help you get ready.

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?
  • I know that you can't predict the future, but am I likely to survive my brain tumor? What can you tell me about the survival rate of people with this diagnosis?
  • 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?
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