Neuroblastoma is cancer that starts
when 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
common solid tumor cancer in babies under age 1. It’s rare in children older than
- Nerve fibers along the spinal
- Clusters of nerve cells (called
ganglia) along the nerve fibers
- Nerve-like cells in the adrenal
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
Neuroblastomas can also form on the
nerve fibers in the abdomen and near the spine in the chest, neck, or lower belly
In most cases, the cancer cells have spread to the bones, lymph
nodes, or both by the time neuroblastoma is diagnosed.
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 of neuroblastoma depend on
the size of the tumor, where it is, and if the cancer has spread. 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
- Trouble passing urine
Symptoms of a tumor in the chest
- A lump you can see or feel
- Swelling in the face, neck, arms, or
- A change in mental state
- Coughing or trouble breathing
- Trouble swallowing
- Drooping eyelids and other eye
- 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
- High blood pressure
- Fast heart rate
- Flushing or redness of skin
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
your child has cancer.
You may take your child to a
healthcare provider because of a lump, swelling, or other change you've noticed. Your
child's 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
Blood tests check for certain substances that could be signs of
disease. Your child may also have urine and blood tests to check for hormones
released by the tumor.
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
X-ray. X-rays may be
done to check your child's chest or bones.
CT scan. X-rays
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. Contrast dye may
be used to get clearer pictures.
MRI scan. An MRI
uses large 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
PET (positron emission
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.
A small amount of radioactive iodine called MIBG is injected
into a vein. It travels through the blood and attaches to cancer cells. Scans may
taken over a few days to see where it collects.
Bone marrow aspiration or
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
taken out with a needle or by making a surgical cut (incision). The removed sample
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
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 decide which treatment options are best. Your
child's doctor will use 1 of these 2 staging systems:
- International Neuroblastoma Staging System (INSS)
- International Neuroblastoma Risk Group Staging System
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 removed by surgery.
- Stages 2 and 3 are more complex. The tumor is harder to
remove and cancer cells may have spread to nearby tissues.
- 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 1 side of the body. There's no cancer in the bones and no more than 10% of
the bone marrow cells are cancer.
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.
- 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 3.
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
- The amount of DNA in the tumor cells (called the DNA
- 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, NSE, and LDH)
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
can understand. Also be sure to ask the healthcare provider if you have any
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.
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.
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 and/or
radiation. This damages the bone marrow. Your child will then get the stem cells to
rebuild the bone marrow.
treatment helps the body's immune system attack cancer cells. It might be used if
there's a high risk of cancer returning.
Retinoid medicine may be used after stem cell transplant if there's
a high risk of cancer returning. It can help reduce the chance of the cancer coming
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
- 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 lessen side effects.
A child may have complications from
the tumor or from treatment. These can include:
- Easy bruising and/or bleeding
- Higher risk of infection
- Hair loss
- Mouth sores
- Nausea and vomiting
- Feeling tired (fatigue)
- Skin changes
- Problems with growth and
- Heart and lung problems
- Changes in sexual development (delayed
- Problems with the ability to have
children (fertility) in the future
- Memory and/or learning problems
- Return of the cancer
- Higher risk of other cancers later in
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
manage side effects. There may be things you can do and medicines you can take to
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.
A child with a neuroblastoma needs
ongoing care. Your child will be seen by oncologists and other healthcare providers
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
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. He or
she will need to balance rest and activity. Encourage your child to be active. This
is good for overall health. And it may help to lessen tiredness.
- Get emotional support for your child.
Find a counselor or child support group can help.
- Make sure your child attends all
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.
- Neuroblastoma is a
cancerous (malignant) tumor that starts in nerve tissue of infants and very young
- 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, and other medicines.
- Continuous follow-up care during
and after treatment is needed
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
- 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
provider after office hours. This is important if your child becomes ill and you have
questions or need advice.