Medulloblastoma
What is medulloblastoma?
Medulloblastoma is a type of brain
cancer. It most often affects children. But in rare cases, it can happen in adults.
These tumors start in the cerebellum, near the back of the brain. The cerebellum
controls balance and coordination.
Any tumor in the brain may press against a part of your brain and
cause problems.
Medulloblastoma is a primary brain
tumor. That means it starts in brain cells. It hasn't spread to the brain from somewhere
else in the body. This type of tumor may spread (metastasize) within the brain and
spinal cord. In rare cases it can spread to the bone marrow, lungs, or other parts of
the body. The outlook for tumors that have spread tends to be worse than for tumors that
stay in one place.
These are fairly fast-growing
tumors. They are grouped into 4 subtypes based on gene changes in the cancer cells:
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WNT. This is the least common
subtype. The cancer cells have mutations of CTNNB1 encoding b-catenin.
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SHH (sonic hedgehog). These cells
have SHH receptor PTCH mutations or SHH inhibitor SUFU mutations. GLI1 and GLI2
amplifications are also possible.
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Group 3. The cells have MYC
amplification and gene expression of photorecepto/GABAergic.
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Group 4. This is the most common sub-group. CDK6
and MYCN are amplified. Neuronal/glutamatergic gene expression is seen.
Talk with your provider if you'd like to learn more about the
subgroup you or your child are in and what the genetics mean for your treatment and
outlook.
What causes medulloblastoma?
A small number of people with
medulloblastoma also have certain inherited disorders. These disorders increase their
risk of getting these tumors, as well as other types of cancer.
These disorders include:
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Gorlin syndrome
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Turcot syndrome
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Cowden syndrome
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Gardner syndrome
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Li-Fraumeni syndrome
But many people who get
medulloblastoma don't have one of these inherited disorders. It's unclear what causes
most of these tumors.
Who is at risk for medulloblastoma?
Medulloblastoma is more common in
children and young adults. In a few people with this condition, there's a link to an
inherited disorder. But in most people, there's no known inherited risk. Researchers are
actively trying to understand the genetics of medulloblastoma in hopes of better
understanding the risk and possible treatments. The risk for medulloblastoma is higher
in:
What are the symptoms of medulloblastoma?
Symptoms of medulloblastoma may
start slowly and get worse as the tumor grows. Headaches are common. Sometimes a brain
tumor may block the normal flow of cerebral spinal fluid (CSF), leading to increased
intracranial pressure. This may cause headache, nausea, vomiting, and dizziness. Because
the tumor is often in the cerebellum, it may affect balance and coordination early on.
Some of the general symptoms of
medulloblastoma include:
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Balance problems or clumsiness
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Changes in thinking ability
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Dizziness
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Double vision or other eye
problems
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Tiredness and lack of energy
(fatigue)
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Headaches
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Nausea and vomiting
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Hearing loss
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Irritability and changed behavior
How is medulloblastoma diagnosed?
Doctors usually take a complete
health history. They will also ask about recent symptoms and past health conditions,
including a family health history. A complete physical exam will be done, including a
nervous system exam. Your doctor may ask you (or your child) to do simple things like
walk, touch your finger to your nose, hold your hands out, or follow a light with your
eyes. If a doctor thinks you may have a brain tumor, imaging tests of the brain will be
needed, such as:
-
MRI scan of the brain and spinal cord. MRIs use
radio waves, magnets, and a computer to make detailed images of the inside of your
body. For this test, you lie still on a table as it passes through a long, tube-like
scanner. If you're not comfortable in small spaces, you may be given a sedative
before the test. A contrast dye may be injected into your blood for this test. It
helps outline the structures in your head so they show up more clearly on the
scans.
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Biopsy. A biopsy is the only sure way to know what
kind of tumor you have. Surgery is done to take out a small piece of the tumor which
is tested right away. If cancer is found, the doctor may then remove as much of the
tumor as possible during the same surgery.
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Spinal tap (lumbar puncture). To do this, a very
thin needle is put through the space between the bones of your lower spine and slid
into the space around your spinal cord. A small amount of the fluid there (called the
CSF or cerebrospinal fluid) is then removed to check it for tumor cells.
You may first see your primary
healthcare provider. He or she may refer you to a doctor that deals with brain
disorders. This can include a:
-
Neurologist. This
specialist diagnoses and treats brain and spinal cord diseases.
-
Neurosurgeon. This is a
specialist who does brain or spinal cord surgery.
-
Neuro-oncologist. This is a
specialist in brain and spinal cord cancers.
How is medulloblastoma treated?
Depending on the type and location
of the tumor, your healthcare team will decide the best treatment plan for you (or your
child). In general, the main treatment in adults is surgery to remove of as much of the
cancer as possible. This both removes the tumor and provides a tissue sample for
testing. Fully removing the tumor increases the chances of a good outcome. After
surgery, you may also have radiation therapy and, in some cases, chemotherapy as well.
Depending on the tumor characteristics, you may have 1 or more of the following
treatments:
-
Surgery to remove of as much of the
tumor as possible (often the main treatment)
-
Radiation therapy to the brain and
spinal cord, including where the tumor was
-
Chemotherapy, in addition to surgery
and radiation
If your intracranial pressure is
high, you may need to have a tube called a shunt inserted. Surgery is done to place the
shunt so that it drains away the excess fluid. This lowers the intracranial pressure.
Follow-up MRI scans will be done regularly to watch your condition.
You will work with your medical
team to decide on the best treatment plan for you.
What are the possible complications of medulloblastoma?
Many people with medulloblastoma
typically have good outcomes. Some people have complications due to the surgery or other
treatments. In some cases, the tumor comes back (recurs) after treatment. To check for
recurrence, you'll likely need follow-up MRI scans in the weeks after surgery and then a
few times a year after that. One possible complication is posterior fossa syndrome. This
a form of short-term (temporary) brain damage, due to surgery. Symptoms may include
problems with language, emotions, and movement. This may last for weeks or years.
Other complications can include:
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Nausea, vomiting, and fatigue, due to radiation therapy or chemotherapy
-
Recurrent tumor
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Side effects of radiation, such as infertility or cognitive changes
Talk with your doctor about the
risk of treatment complications (both short- and long-term), your overall outlook
(prognosis), and the chances of the tumor returning.
Key points about medulloblastoma
-
Medulloblastoma is the most common
brain cancer in children. It's rare in adults.
-
These tumors start in the cerebellum, the part of the brain that
controls your balance and coordination.
-
Symptoms often include headache,
nausea, and vomiting, especially in the morning.
-
You may need surgery to remove as much
of the tumor as possible.
-
You may need radiation therapy and
sometimes chemotherapy after surgery.
Next steps
Tips to help you get the most from a visit to your healthcare provider:
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Know the reason for your visit and what you want to happen.
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Before your visit, write down questions you want answered.
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Bring someone with you to help you ask
questions and remember what your healthcare provider tells you.
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At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
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Know why a new medicine or treatment
is prescribed, and how it will help you. Also know what the side effects are.
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Ask if your condition can be treated in other ways.
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Know why a test or procedure is recommended and what the results could mean.
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Know what to expect if you do not take the medicine or have the test or procedure.
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If you have a follow-up appointment, write down the date, time, and purpose for that visit.
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Know how you can contact your healthcare provider if you have questions.
Online Medical Reviewer:
Kim Stump-Sutliff RN MSN AOCNS
Online Medical Reviewer:
Lu Cunningham
Online Medical Reviewer:
Richard LoCicero MD
Date Last Reviewed:
11/1/2016
© 2000-2019 The StayWell Company, LLC. 800 Township Line Road, Yardley, PA 19067. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.