Neuroblastoma
Overview
Neuroblastoma is cancer that starts
when immature nerve cells in the nervous system grow out of control. These cells are
called neuroblasts. Over time, the out-of-control cells form a tumor. Neuroblastoma
is the most common solid tumor cancer in babies under age 1. Most neuroblastomas are
found in children younger than 5 years. It’s rare in children older than age 10.
Neuroblastoma affects:
- Nerve fibers along the spinal
cord
- Clusters of nerve cells (called
ganglia) along the nerve fibers
- Nerve-like cells in the adrenal
glands
In most cases, neuroblastoma starts
in the adrenal glands. These glands are on the top of both kidneys. They make hormones
that help control things like heart rate, blood pressure, and the body's stress
response.
Neuroblastomas can also form on the
nerve fibers in the abdomen and near the spine in the chest, neck, or lower belly
(pelvis).
In most cases, the cancer cells have spread to the bones, lymph
nodes, or both by the time neuroblastoma is diagnosed.
Causes
Cancer is caused by changes (mutations) in the DNA of the cells. In
most children, this happens by chance. A very small number of children inherit the
changed genes from their parents.
Symptoms
Symptoms of neuroblastoma depend on
the size of the tumor, where it is, if the cancer has spread, and if the neuroblastoma
cells make hormones. They tend to be different in each child.
Symptoms of a tumor in the belly
(abdomen) can include:
- A lump you can see or feel
- Swelling in the abdomen
- Loss of appetite
- Weight loss
- Swelling of the legs
- Swelling of the scrotum
- Feeling full
- Pain
- Constipation
- Trouble passing urine
Symptoms of a tumor in the chest
can include:
- A lump you can see or feel
- Swelling in the face, neck, arms, or
chest (change in skin color to bluish-red)
- Headache
- Dizziness
- A change in mental state
- Coughing or trouble breathing
- Trouble swallowing
- Drooping eyelids and other eye
changes
- Changes in feeling or movement of the
arms or legs
Symptoms of a neuroblastoma that
has spread to other parts of the body can include:
- Enlarged lymph nodes
- Bone pain or limping
- Weakness, numbness, or inability to
move arms or legs
- Bruising around the eyes
- Bulging eyes
- Enlarged liver
- Bluish bumps on the skin
- Lumps on the head
- Feeling tired or weak
- Frequent infections
- Easy bruising or bleeding
A neuroblastoma may release
hormones. This is called paraneoplastic syndrome. It can cause symptoms, such as:
- Constant watery diarrhea
- Fever
- High blood pressure
- Fast heart rate
- Flushing or redness of skin
- Sweating
A neuroblastoma can also cause
opsoclonus-myoclonus-ataxia syndrome. This can lead to symptoms, such as:
- Quick eye movements
- Muscle twitching or spasms
- Trouble with coordination
- Trouble speaking
Many of these symptoms can be
caused by other health problems. Still, it's important to take your child to a
healthcare provider if you notice these symptoms. Only a healthcare provider can tell
if
your child has cancer.
Diagnosis
You may take your child to a
healthcare provider because of a lump, swelling, or other change you've noticed. Your
child's healthcare provider will ask about your child's medical history and symptoms.
A
physical exam will be done. Your child may be referred to a specialist in diagnosing
and
treating cancer in children (called pediatric oncologist).
If a neuroblastoma is suspected,
tests will be needed to learn more:
-
Blood and urine
tests.
Blood tests check for certain substances that could be signs of
disease and to check your child's overall health and organ function. Your child may
also have urine and blood tests to check for hormones released by the tumor. These
hormones are called catecholamines (such as epinephrine and norepinephrine).
Catecholamine metabolites (substances made when catecholamines break down) are
usually found in the urine of children with neuroblastoma.
-
Ultrasound exam
(US).
Sound waves and a computer are used to create images of the inside of
the body. An ultrasound may be done to check your child's belly (abdomen) or
kidneys.
-
X-ray. X-rays may be
done to check your child's chest or bones.
-
CT scan. X-rays taken
from different angles and a computer are used to make 3-D images of the inside of
your child's body. Scans of the belly (abdomen), lower belly (pelvis), or chest may
be done. A contrast medium may be given before the scan to get clearer pictures.
-
MRI scan. An MRI
uses large, powerful magnets, radio waves, and a computer to make detailed
pictures of the inside of the body. This test is used to check for tumors in the
brain and spinal cord. A contrast medium may be given before the scan to get clearer
pictures.
-
PET (positron emission
tomography) scan.
For this test, a radioactive sugar is put right into
the bloodstream. Cancer cells use the sugar faster than normal cells, so the sugar
collects in cancer cells. A special camera is then used to see where the radioactive
sugar is in the body. A PET scan can sometimes spot cancer cells in different parts
of the body, even when they can’t be seen with other tests. This test is often used
in combination with a CT scan. This is called a PET/CT scan.
-
MIBG (meta-iodobenzylguanidine)
scan.
Neuroblastoma cells absorb MIBG (a protein). A small amount of
radioactive iodine is linked to MIBG and is injected into a vein. It travels through
the blood and attaches to cancer cells. Scans may be taken over a few days to see
where it collects. Regular iodine is taken by mouth for several days before the scan.
This is done to protect your child's thyroid gland from absorbing the radioactive
iodine in MIBG.
-
Bone marrow aspiration or
biopsy.
Bone marrow is found in the center of some bones. It’s where
blood cells are made. A small amount of bone marrow fluid may be taken out with a
needle. This is called aspiration. Or solid bone marrow tissue may be taken with a
bigger needle. This is called a core biopsy. Bone marrow is usually taken from the
back of the pelvic bone. This test may be done to see if cancer cells have reached
the bone marrow.
-
Tumor biopsy. This is
when a small piece (called a sample) of the tumor is taken out for testing. It may
be
taken out with a needle or by making a surgical cut (incision). The removed sample
is
tested for cancer cells. A biopsy is needed to diagnose neuroblastoma. An imaging
test, like an ultrasound or CT scan may be used to guide the needle during a
biopsy.
Staging neuroblastoma
Once neuroblastoma has been
diagnosed, a stage is assigned. The stage is how much cancer there is and if and
where it has spread. Staging is used to guide treatment options. Your child's
healthcare provider will use one of these two staging systems:
- International Neuroblastoma Staging System (INSS)
- International Neuroblastoma Risk Group Staging System
(INRGSS)
The
INSS system divides tumors into stages 1 through 4.
It’s assigned after surgery has been done to remove the tumor.
- Stage 1 is early cancer. It's only in the place where it
first started and only on one side of the body. This means the tumor can be
fully seen and completely removed by surgery. Lymph nodes near the tumor do not
have cancer. Lymph nodes attached to the tumor after surgery may or may not
have cancer.
- Stage 2:
- Stage 2A. The cancer has not spread to nearby lymph nodes. It's only
located in the area where it started on one side of the body. All of the
tumor that can be seen can't be completely removed during surgery.
- Stage 2B. The cancer has spread to nearby lymph nodes. It's only located
in the area where it started on one side of the body and may or may not
be completely removed during surgery.
- Stage 3: The cancer has not spread to distant parts of the
body. One of the following is true:
- The cancer can't be fully removed with surgery. It has spread to the
other side of the body and may have spread to nearby lymph nodes.
- The cancer is on one side of the body and has spread to lymph nodes on
the other side.
- The cancer is in the middle of the body and has spread to tissues or
lymph nodes on both sides of the body. The cancer can't be fully removed
with surgery.
- Stage 4 means cancer cells from the tumor have spread to
other parts of the body far from the primary (first) tumor. They may be in
distant lymph nodes, the bone marrow, skin, liver, or bones.
- Stage 4S is only used in infants under age 1. The cancer
may have spread to lymph nodes, the liver, skin, or bone marrow, but it's only
on one side of the body. There's no cancer in the bones and no more than 10% of
the bone marrow cells are cancer.
The
INRGSS system divides tumors into 4 stages labeled
L1, L2, M, and MS. Surgery does not need to be done before assigning one of these
stages. They're based on how the tumor looks on imaging scans and biopsies. These
stages are used to predict how much of the tumor can be taken out with surgery.
- The L groups means the cancer is only in one part of the
body and has not spread far from where it first started. Stage L1 has no IDRFs
(image-defined risk factors) found on imaging scans. Stage L2 does have IDRFs
found on imaging scans (such as a CT scan).
- The M group means it has spread beyond where it first
started to distant organs.
- MS is used in children younger than 18 months with cancer
that has spread to the skin, liver, less than 10% of the bone marrow, or all
three. There's no sign of cancer in the bones.
The healthcare team also looks
at prognostic factors to get an idea of how your child’s neuroblastoma will likely
respond to treatment. These factors include:
- Where the main tumor is
- Gene and chromosome changes in the
tumor cells
- The amount of DNA in the tumor cells (called the DNA
index)
- Tumor histology, which is how different the cancer cells look
compared to normal cells and tissues
- The presence and amount of certain receptors on the cancer
cells (called neurotrophin receptors)
- The age of your child
- Lab test results that measure the levels of certain
substances in your child's blood (like ferritin, neuron-specific enolase, and
lactate dehydrogenase)
Your child’s healthcare team
will tell you more about the stage of your child’s cancer and what the prognostic
factors mean. The stages of neuroblastoma are very complex. Be sure to ask the
healthcare provider to explain the details of your child's cancer to you in a way
you
can understand. Also be sure to ask the healthcare provider if you have any
questions.
Treatment
Treatment will depend on the stage
and other factors. Neuroblastomas can be treated with any of these:
-
Surgery. Surgery is
often done to remove as much of the tumor as possible (called resection). It may not
be an option if the cancer has spread.
-
Chemotherapy. These are
strong drugs that kill cancer cells or stop them from growing. They may be given into
the vein (IV), injected as a shot, or taken by mouth. Chemo might be used before or
after surgery. Or it may be the main treatment if surgery can't be done.
-
Radiation
therapy.
High-energy X-rays or other types of energy are used to kill cancer
cells or stop them from growing. Radiation beams are aimed at the tumor from a large
machine. This is called external beam radiation.
-
High-dose chemotherapy/radiation
with a stem cell transplant.
Young blood cells (stem cells) are taken
from the child or from a donor. This is followed by high doses of chemotherapy,
radiation, or both. This damages the bone marrow. Your child will then get the stem
cells to rebuild the bone marrow.
-
Immunotherapy. This
treatment helps the body's immune system attack cancer cells. A substance called GD2
may be present in large amounts on neuroblastoma cells. Anti-GD2 monoclonal
antibodies might be used if there's a high risk of cancer returning.
-
Retinoid
therapy.
Retinoid medicine may be used for 6 months after stem cell
transplant if there's a high risk of cancer returning. It can help reduce the chance
of the cancer coming back.
-
Supportive
care.
Treatment can cause side effects. Medicines and other treatments can
be used for pain, fever, infection, and nausea and vomiting.
-
Clinical trials. Ask
your child's healthcare provider if there are any treatments being tested that may
work well for your child. Clinical trials allow your child to get the best treatment
available now, and maybe new treatments that are expected to be better. Most children
with cancer are treated as part of a clinical trial.
With any cancer, how well a child is expected to recover (prognosis) varies. Keep
in mind:
- Getting medical treatment right away
is important for the best outcome.
- Ongoing follow-up care during and after treatment is needed.
- New treatments are being tested to
improve outcome and to reduce side effects.
Complications
A child may have complications from
the tumor or from treatment. These can include:
- Easy bruising, bleeding, or both
- Higher risk of infection
- Hair loss
- Mouth sores
- Nausea and vomiting
- Diarrhea
- Feeling tired (fatigue)
- Skin changes
- Problems with growth and
development
- Heart and lung problems
- Infusion reactions
- Eye and vision problems
- Changes in sexual development (delayed
puberty)
- Problems with the ability to have
children (fertility) in the future
- Memory, learning problems, or both
- Return of the cancer
- Higher risk of other cancers later in
life
Talk with your child's healthcare provider about side effects linked
with their treatment. Tell them about any changes you notice. There are often ways
to
manage side effects. There may be things your child can do and medicines they can
take
to help prevent or control many treatment side effects.
Most side effects get better and go away over time after treatment
ends. But some can last the rest of your child's life. Talk to your child's treatment
team about what you can expect.
Living with
A child with a neuroblastoma needs
ongoing care. Your child will be seen by oncologists and other healthcare providers
to
treat any late effects of treatment and to watch for signs or symptoms of the tumor
returning. Imaging scans and other tests will be done.
Your child may need therapy to help
with movement and muscle strength. This may be done by physical and occupational
therapists.
You can help your child manage
treatment in many ways. For instance:
- Your child may have trouble eating. A
dietitian may be able to help.
- Your child may be very tired. They
will need to balance rest and activity. Encourage your child to be active. This is
good for overall health. And it may help to reduce tiredness. Ask your child's
healthcare provider what exercises are safe for your child.
- Get emotional support for your child.
Find a counselor, psychologist, or child support group that can help.
- Make sure your child attends all
follow-up appointments.
When to Call a Healthcare Provider
Your child's healthcare provider
will talk with you about when to call. You may be told to call if your child has:
- Symptoms that get worse
- New symptoms
- Signs of an infection, such as fever
- Side effects from treatment that
affect your child's daily routine or don't get better with treatment
Ask your child's provider what signs to watch for and when to call.
Know how to get help after office hours and on weekends and holidays.
Key Points
- Neuroblastoma is a
cancerous (malignant) tumor that starts in nerve tissue of infants and very young
children.
- The symptoms of neuroblastoma vary a
lot depending on the size of the tumor, where it is, and whether it has spread.
Common symptoms are a lump or swelling.
- Neuroblastoma is diagnosed with blood and urine tests, imaging tests, and biopsy.
- Treatment of neuroblastoma may include
surgery, chemotherapy, radiation therapy, high-dose chemotherapy/radiation with stem
cell transplant, immunotherapy, and other medicines.
- Continuous follow-up care during
and after treatment is needed
Next Steps
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- 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 for your child.
- Know why a new medicine or treatment
is prescribed and how it will help your child. Also know what the side effects
are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
- Know how you can contact your child’s
healthcare provider after office hours. This is important if your child becomes ill
and you have questions or need advice.