Central nervous system embryonal tumors begin in
embryonic (fetal) cells in the brain and spinal cord.
Central nervous system (CNS) embryonal tumors form in
brain cells when the fetus is beginning to develop. The tumors may be benign
(not cancer) or malignant (cancer). Most CNS embryonal tumors in children are
malignant.
Although cancer is rare in children, brain tumors are
the third most common type of childhood cancer, after leukemia and lymphoma.
This summary discusses the treatment of primary brain tumors (tumors that begin
in the brain). The treatment of metastatic brain tumors, which begin in other
parts of the body and spread to the brain, is not discussed in this summary.
Brain tumors can occur in both children and adults;
however, treatment for children may be different than treatment for adults.
CNS embryonal tumors may form in different areas of the brain.
Childhood brain tumors are named based on the type of
cell they formed in and where the tumor first formed in the brain. There are 6
different types of CNS embryonal tumors:
Medulloblastoma
Medulloblastomas are fast-growing tumors that form in
brain cells in the cerebellum. The cerebellum is at the lower back part of the
brain between the cerebrum and the brain stem. The cerebellum controls movement,
balance, and posture. Sometimes medulloblastoma spreads to the bone, bone
marrow, lymph nodes, liver, or lung.
Pineoblastoma
Pineoblastoma is a fast-growing tumor that forms in
brain cells in or near the pineal gland. The pineal gland is a tiny organ in the
brain that makes melatonin, a substance that helps control our sleep cycle.
Pineal parenchymal tumors
Pineal parenchymal tumors form in pineocytes, a type
of cell in the pineal gland. These tumors may be slow or fast-growing.
Supratentorial primitive neuroectodermal tumors
Supratentorial primitive neuroectodermal tumors are
fast-growing tumors that form in brain cells in the cerebrum. The cerebrum is at
the top of the head and is the largest part of the brain. The cerebrum controls
thinking, learning, problem-solving, emotions, speech, reading, writing, and
voluntary movement.
Medulloepithelioma
Medulloepitheliomas are fast-growing tumors that form
in brain cells that line tubelike spaces in the brain and spinal cord. These
rare tumors are most common in infants and young children.
Ependymoblastoma
Ependymoblastomas are fast-growing tumors that form in
brain cells lining the fluid -filled spaces in the brain and spinal cord. These
rare tumors are most common in infants and young children.
Childhood CNS atypical teratoid/rhabdoid tumor is also
a type of embryonal tumor, but it is treated differently than other childhood
CNS embryonal tumors.
Certain genetic conditions increase the risk of
childhood CNS embryonal tumors.
Anything that increases the risk of getting a disease
is called a risk factor. Having a risk factor does not mean that you will get
cancer; not having risk factors doesn’t mean that you will not get cancer.
Parents who think their child may be at risk should discuss this with the
child's doctor. Risk factors for CNS embryonal tumors include having the
following inherited diseases:
- Li-Fraumeni syndrome.
- Nevoid basal cell carcinoma (Gorlin) syndrome.
- Turcot syndrome.
In most cases, the cause of CNS embryonal tumors is not known.
The symptoms of childhood CNS embryonal tumors are not the same in every child.
The following symptoms and others may be caused by a
CNS embryonal tumor. Symptoms vary depending on the child's age and where the
tumor is located. Other conditions may cause the same symptoms. A doctor should
be consulted if any of the following problems occur:
- Loss of balance, trouble walking, worsening handwriting, or slow speech.
- Morning headache or headache that goes away after vomiting.
- Nausea and vomiting.
- General weakness or weakness on one side of the face.
- Unusual sleepiness or change in energy level.
- Change in personality or behavior.
- Unexplained weight loss or weight gain.
An infant or young child may be irritable or grow
slowly, and may not eat well or meet developmental milestones such as sitting,
walking, and talking in sentences.
Tests that examine the brain and spinal cord are
used to detect (find) childhood CNS embryonal tumors.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general
signs of health, including checking for signs of disease, such as lumps or
anything else that seems unusual. A history of the patient’s health habits
and past illnesses and treatments will also be taken.
- Neurological exam: A series of questions and tests to check the
brain, spinal cord, and nerve function. The exam checks a patient's mental
status, coordination, and ability to walk normally, and how well the
muscles, senses, and reflexes work. This may also be called a neuro exam or
a neurologic exam.
- CT scan (CAT scan): A procedure that makes a series of detailed
pictures of areas inside the body, taken from different angles. The pictures
are made by a computer linked to an x-ray machine. A dye may be injected
into a vein or swallowed to help the organs or tissues show up more clearly.
This procedure is also called computed tomography, computerized tomography,
or computerized axial tomography.
- MRI (magnetic resonance imaging) of the brain and spinal cord with
gadolinium: A procedure that uses a magnet, radio waves, and a computer to
make a series of detailed pictures of areas inside the brain and spinal
cord. A substance called gadolinium is injected into a vein. The gadolinium
collects around the cancer cells so they show up brighter in the picture.
This procedure is also called nuclear magnetic resonance imaging (NMRI).
Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI
scan to look at the chemical makeup of the brain tissue.
- Lumbar puncture: A procedure used to collect cerebrospinal fluid
from the spinal column. This is done by placing a needle into the spinal
column. This procedure is also called an LP or spinal tap.
- SPECT scan (single photon emission computed tomography): A
procedure that uses a special camera linked to a computer to make a
3-dimensional (3-D) picture of the brain. A small amount of a radioactive
substance is injected into a vein or inhaled through the nose. As the
substance travels through the blood, the camera rotates around the head and
takes pictures of the brain. There will be increased blood flow and more
chemical reactions (metabolism) in areas where cancer cells are growing.
These areas will show up brighter in the picture. This procedure may be done
just before or after a CT scan.
- PET scan (positron emission tomography scan): A procedure to find
malignant tumor cells in the body. A small amount of radioactive glucose
(sugar) is injected into a vein. The PET scanner rotates around the body and
makes a picture of where glucose is being used in the brain. Malignant tumor
cells show up brighter in the picture because they are more active and take
up more glucose than normal cells do.
Childhood CNS embryonal tumors are usually diagnosed and removed in surgery.
If doctors think your child may have a CNS embryonal
tumor, a biopsy may be done to remove a sample of tissue. For brain tumors, the
biopsy is done by removing part of the skull and using a needle to remove a
sample of tissue. Sometimes, a computer-guided needle is used to remove a sample
of tissue. A pathologist views the tissue under a microscope to look for cancer
cells. If cancer cells are found, the doctor may remove as much tumor as safely
possible during the same surgery.
The following tests may be done on the sample of tissue that is removed:
- Immunohistochemistry study: A laboratory test in which a substance
such as an antibody, dye, or radioisotope is added to a sample of cancer
tissue to test for certain antigens. This type of study is used to tell the
difference between different types of cancer.
- Light and electron microscopy: A laboratory test in which cells in a
sample of tissue are viewed under regular and high-powered microscopes to
look for certain changes in the cells.
- Cytogenetic analysis: A laboratory test in which cells in a sample
of tissue are viewed under a microscope to look for certain changes in the
chromosomes.
Some pineoblastoma and pineal parenchymal tumors are diagnosed by brain imaging tests.
Sometimes, it is not possible to do a safe biopsy or completely remove the tumor in surgery,
because of where it is in the brain. These tumors are diagnosed based on the results of imaging tests and other procedures.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
- The age of the child when the tumor is found.
- The type of tumor and where it is in the brain.
- Whether there is tumor left after surgery.
- Whether the cancer has spread within the brain and spinal cord or to
other parts of the body, such as the bones.
- Whether the tumor has just been diagnosed or has recurred (come back).
Staging Childhood Central Nervous System Embryonal Tumors
Treatment is based on the child’s risk group.
Staging is the process used to find out how much cancer there is and if cancer has spread.
It is important to know the stage in order to plan treatment.
There is no standard staging system for childhood central nervous system (CNS) embryonal tumors. Instead, treatment depends on the child’s risk group:
Average risk
Childhood CNS embryonal tumors are called average risk
when all of the following are true:
- The child is older than 3 years of age.
- The tumor is at the very back of the brain.
- All of the tumor was removed by surgery or there was only a very small
amount remaining.
- The cancer has not spread to other parts of the body.
High risk
Childhood CNS embryonal tumors are called high risk if any of the following are true:
- The child is 3 years of age or younger.
- The tumor is not at the very back of the brain.
- Some of the tumor was not removed by surgery.
- The cancer has spread to other parts of the body.
In general, cancer is more likely to recur (come back) in patients in the high-risk group.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and
travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and
travels through the blood to other places in the body.
When cancer cells break away from the primary
(original) tumor and travel through the lymph or blood to other places in the
body, another (secondary) tumor may form. This process is called metastasis. The
secondary (metastatic) tumor is the same type of cancer as the primary tumor.
For example, if breast cancer spreads to the bones, the cancer cells in the
bones are actually breast cancer cells. The disease is metastatic breast cancer,
not bone cancer.
The information from tests and procedures done to detect (find) childhood CNS embryonal
tumors is used to plan cancer treatment.
Some of the tests used to detect childhood CNS
embryonal tumors are repeated after the tumor is removed by surgery. This is to
find out how much tumor remains after surgery. Other tests and procedures may be
done to find out if the cancer has spread:
- Lumbar puncture: A procedure used to collect cerebrospinal fluid
from the spinal column. This is done by placing a needle into the spinal
column. This procedure is also called an LP or spinal tap.
- Bone marrow aspiration and biopsy: The removal of bone marrow,
blood, and a small piece of bone by inserting a hollow needle into the
hipbone or breastbone. A pathologist views the bone marrow, blood, and bone
under a microscope to look for signs of cancer.
- Bone scan: A procedure to check if there are rapidly dividing cells,
such as cancer cells, in the bone. A very small amount of radioactive
material is injected into a vein and travels through the bloodstream. The
radioactive material collects in the bones and is detected by a scanner.
Recurrent Childhood Central Nervous System Embryonal Tumors
A recurrent childhood central nervous system (CNS)
embryonal tumor is a tumor that recurs (comes back) after being treated.
Childhood CNS embryonal tumors most often recur within 18 months after treatment
but may come back many years later. Recurrent childhood CNS embryonal tumors may
come back in the same place as the original tumor or in a different place in the
brain or spinal cord.
Treatment Option Overview
There are different types of treatment for children who have CNS embryonal tumors.
Different types of treatment are available for
children with central nervous system (CNS) embryonal tumors. Some treatments are
standard (the currently used treatment), and some are being tested in clinical
trials. A treatment clinical trial is a research study meant to help improve
current treatments or obtain information on new treatments for patients with
cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a
clinical trial should be considered. Some clinical trials are open only to
patients who have not started treatment.
Children who have CNS embryonal tumors should have
their treatment planned by a team of health care providers who are experts in
treating brain tumors in children.
Treatment will be overseen by a pediatric oncologist,
a doctor who specializes in treating children with cancer. The pediatric
oncologist works with other pediatric health care providers who are experts in
treating children with brain tumors and who specialize in certain areas of
medicine. These may include the following specialists:
- Neurosurgeon
- Neurologist
- Neuropathologist
- Neuroradiologist
- Rehabilitation specialist
- Radiation oncologist
- Psychologist
Childhood brain tumors may cause symptoms that begin before diagnosis and continue for
months or years.
Symptoms caused by the tumor may begin before
diagnosis. These symptoms may continue for months or years. It is important to
talk with your child's doctors about symptoms caused by the tumor that may
continue after treatment.
Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during
or after treatment and continue for months or years are called late effects.
Late effects of cancer treatment may include the following:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
Children diagnosed with medulloblastoma may have cerebellar mutism syndrome after surgery.
Symptoms of this syndrome include the following:
- Not being able to speak.
- Trouble swallowing and eating.
- Loss of balance, trouble walking, and worsening handwriting.
- Loss of muscle tone.
- Mood swings and changes in personality.
Some late effects may be treated or controlled. It is
important to talk with your child's doctors about the effects cancer treatment
can have on your child.
Three types of standard treatment are used:
Surgery
Surgery is used to diagnose and treat a childhood CNS
embryonal tumor as described in this summary.
Even if the doctor removes all the cancer that can be
seen at the time of the surgery, some patients may be given chemotherapy or
radiation therapy after surgery to kill any cancer cells that are left.
Treatment given after the surgery to lower the risk that the cancer will come
back is called adjuvant therapy.
Radiation therapy
Radiation therapy is a cancer treatment that uses
high-energy x-rays or other types of radiation to kill cancer cells or keep them
from growing. There are two types of radiation therapy. External radiation
therapy uses a machine outside the body to send radiation toward the cancer.
Internal radiation therapy uses a radioactive substance sealed in needles,
seeds, wires, or catheters that are placed directly into or near the cancer.
Radiation therapy to the brain can affect growth and
development in young children. For this reason, clinical trials are studying new
ways of giving radiation that may have fewer side effects than standard methods.
For childhood CNS embryonal tumors, radiation therapy may be given in the
following ways:
- Conformal radiation therapy uses a computer to make a 3-dimensional
(3-D) picture of the tumor and the radiation beams are shaped to fit the
tumor. This allows a high dose of radiation to reach the tumor and causes
less damage to normal tissue around the tumor.
- Stereotactic radiation therapy uses a rigid head frame attached to the
skull to aim radiation directly to a tumor, causing less damage to normal
tissue around the tumor. The total dose of radiation is divided into several
smaller doses given over several days. This procedure is also called
stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
The way the radiation therapy is given depends on the type of cancer being treated.
Because radiation therapy can affect growth and brain
development in young children, especially children who are three years old or
younger, chemotherapy may be given to delay or reduce the need for radiation therapy.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to
stop the growth of cancer cells, either by killing the cells or by stopping them
from dividing. When chemotherapy is taken by mouth or injected into a vein or
muscle, the drugs enter the bloodstream and can reach cancer cells throughout
the body (systemic chemotherapy). When chemotherapy is placed directly into the
spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly
affect cancer cells in those areas (regional chemotherapy). Combination
chemotherapy is treatment using more than one anticancer drug. The way the
chemotherapy is given depends on the type of cancer being treated.
Anticancer drugs given by mouth or vein to treat
central nervous system tumors cannot cross the blood-brain barrier and enter the
fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is
injected into the fluid-filled space to kill cancer cells that may have spread
there. This is called intrathecal chemotherapy.
New types of treatment are being tested in clinical trials.
This section describes treatments that are being
studied in clinical trials. It may not mention every new treatment being
studied. Information about clinical trials is available from the NCI Web site.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a
way of giving high doses of chemotherapy and replacing blood -forming cells
destroyed by the cancer treatment. Stem cells (immature blood cells) are removed
from the blood or bone marrow of the patient or a donor and are frozen and
stored. After the chemotherapy is completed, the stored stem cells are thawed
and given back to the patient through an infusion. These reinfused stem cells
grow into (and restore) the body’s blood cells.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may
be the best treatment choice. Clinical trials are part of the cancer research
process. Clinical trials are done to find out if new cancer treatments are safe
and effective or better than the standard treatment.
Many of today's standard treatments for cancer are
based on earlier clinical trials. Patients who take part in a clinical trial may
receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help
improve the way cancer will be treated in the future. Even when clinical trials
do not lead to effective new treatments, they often answer important questions
and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have
not yet received treatment. Other trials test treatments for patients whose
cancer has not gotten better. There are also clinical trials that test new ways
to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the
cancer or to find out the stage of the cancer may be repeated. Some tests will
be repeated in order to see how well the treatment is working. Decisions about
whether to continue, change, or stop treatment may be based on the results of
these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time
to time after treatment has ended. The results of these tests can show if your
child's condition has changed or if the brain tumor has recurred (come back). If
the tumor recurs in the brain, a biopsy may also be done to find out if it is
made up of dead tumor cells or if new cancer cells are growing. These tests are
sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Central Nervous System Embryonal Tumors
For some types or stages of cancer, there may not be
any trials listed. Check with your doctor for clinical trials that are not
listed here but may be right for you.
Newly Diagnosed Childhood Medulloblastoma
In newly diagnosed childhood medulloblastoma, the
tumor itself has not been treated. The child may have received drugs or
treatment to relieve symptoms caused by the tumor.
Average risk
Standard treatment of average-risk childhood
medulloblastoma is usually surgery followed by radiation therapy to the brain
and spinal cord. Sometimes chemotherapy is given at the same time as radiation
therapy or after radiation therapy.
Clinical trials are studying new combinations of radiation therapy and chemotherapy.
High risk
Standard treatment of high-risk childhood
medulloblastoma is usually surgery followed by radiation therapy to the brain
and spinal cord. Sometimes chemotherapy is given at the same time as radiation
therapy or after radiation therapy.
Clinical trials are studying new combinations of radiation therapy and chemotherapy.
Children 3 years old or younger
Standard treatment of childhood medulloblastoma in
children 3 years old or younger is usually surgery followed by chemotherapy.
Other treatments may include the following:
- Surgery followed by combination chemotherapy.
- Surgery followed by high-dose chemotherapy with stem cell transplant.
- Surgery followed by combination chemotherapy and radiation therapy to
the area where the tumor was removed.
Treatment of medulloblastoma in children 3 years old
or younger is often within a clinical trial. Clinical trials are studying new
combinations of chemotherapy with stem cell transplant.
Newly Diagnosed Childhood Pineoblastoma and Pineal Parenchymal Tumors
In newly diagnosed childhood pineoblastoma or pineal
parenchymal tumors, the tumor itself has not been treated. The child may have
received drugs or treatment to relieve symptoms caused by the tumor.
Children older than 3 years
Standard treatment of childhood pineoblastoma and
pineal tumors in children older than 3 years is usually surgery. It is usually
not possible to remove all of the tumor because of where it is in the brain.
Surgery is usually followed by radiation therapy to the brain and spinal cord.
Sometimes chemotherapy is given at the same time as radiation therapy or after radiation therapy.
Clinical trials are studying new treatments for high-risk childhood pineoblastoma, including combinations of chemotherapy, radiation therapy, and stem cell transplant.
Children 3 years old or younger
Treatment of pineoblastoma and pineal tumors in
children 3 years old or younger may include surgery followed by chemotherapy.
Radiation therapy may be given when the child is older. High-dose chemotherapy
with stem cell transplant has been used.
Newly Diagnosed Childhood Supratentorial Primitive Neuroectodermal Tumors
In newly diagnosed childhood supratentorial primitive
neuroectodermal tumors, the tumor itself has not been treated. The child may
have received drugs or treatment to relieve symptoms caused by the tumor.
Children older than 3 years
Standard treatment of supratentorial neuroectodermal
tumors in children older than 3 years is usually surgery followed by radiation
therapy to the brain and spinal cord. Sometimes chemotherapy is given at the
same time as radiation therapy or after radiation therapy.
Children 3 years old or younger
Standard treatment of supratentorial neuroectodermal
tumors in children 3 years old or younger is usually surgery followed by
chemotherapy. Other treatments may include the following:
- Surgery followed by combination chemotherapy.
- Surgery followed by high-dose chemotherapy with stem cell transplant.
- Surgery followed by combination chemotherapy and radiation therapy to
the area where the tumor was removed.
Treatment of supratentorial primitive neuroectodermal tumors in children 3 years old or younger
is often within a clinical trial. Clinical trials are studying new combinations of chemotherapy
with stem cell transplant.
Newly Diagnosed Childhood Medulloepithelioma and Ependymoblastoma
In newly diagnosed childhood medulloepithelioma and
ependymoblastoma, the tumor itself has not been treated. The child may have
received drugs or treatment to relieve symptoms caused by the tumor.
Children older than 3 years
Standard treatment of childhood medulloepithelioma or
ependymoblastoma in children older than 3 years is usually surgery followed by
radiation therapy to the brain and spinal cord. Sometimes chemotherapy is given
at the same time as radiation therapy or after radiation therapy.
Children 3 years old or younger
Standard treatment of childhood medulloepithelioma or
ependymoblastoma in children 3 years old or younger is usually surgery followed
by chemotherapy. Other treatments may include the following:
- Surgery followed by combination chemotherapy.
- Surgery followed by high-dose chemotherapy with stem cell transplant.
- Surgery followed by combination chemotherapy and radiation therapy to
the area where the tumor was removed.
Treatment of childhood medulloepithelioma or ependymoblastoma in children 3 years old or
younger is often within a clinical trial.
Recurrent Childhood Central Nervous System Embryonal Tumors
Treatment of recurrent childhood CNS embryonal tumors may include the following:
- For patients who previously received radiation therapy and chemotherapy,
treatment may be chemotherapy and/or stereotactic radiation therapy.
- For infants and young children who previously received chemotherapy only
and have a local recurrence, treatment may be chemotherapy with radiation
therapy to the tumor and the area close to it.
- For patients who previously received radiation therapy, high-dose
chemotherapy and stem cell transplant may be used.
Check for U.S. clinical trials from NCI's PDQ Cancer
Clinical Trials Registry that are now accepting patients with childhood central
nervous system embryonal tumor. For more specific results, refine the search by
using other search features, such as the location of the trial, the type of
treatment, or the name of the drug. General information about clinical trials is
available from the NCI Web site.
For more information from the NCI, please write to this address:
NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322
U.S. residents may call the National Cancer Institute's (NCI's) Cancer
Information Service toll-free at 1.800.4-CANCER (1.800.422.6237) Monday through
Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY
equipment may call 1.800.332.8615. Information about ongoing clinical trials is available from the NCI Web site.
Source: National Institutes of Health; National Cancer Institute
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 8/12/2009...#6136
