Neuroblastoma is a cancer that develops from immature nerve cells found

in several areas of the body.

Neuroblastoma most commonly arises in and around the adrenal glands,
which have similar origins to nerve cells and sit atop the kidneys. However,
neuroblastoma can also develop in other areas of the abdomen and in the
chest, neck and near the spine, where groups of nerve cells exist.
Neuroblastoma most commonly affects children age 5 or younger, though it
may rarely occur in older children.
Some forms of neuroblastoma go away on their own, while others may
require multiple treatments. Your child's neuroblastoma treatment options
will depend on several factors.
Symptoms

Adrenal glands
Signs and symptoms of neuroblastoma vary depending on what part of the
body is affected.

Neuroblastoma in the abdomen the most common form may cause

signs and symptoms such as:

Abdominal pain

A mass under the skin that isn't tender when touched

Changes in bowel habits, such as diarrhea or constipation

Neuroblastoma in the chest may cause signs and symptoms such as:

Wheezing

Chest pain

Changes to the eyes, including drooping eyelids and unequal pupil

size
Other signs and symptoms that may indicate neuroblastoma include:

Lumps of tissue under the skin

Eyeballs that seem to protrude from the sockets (proptosis)

Dark circles, similar to bruises, around the eyes

Back pain

Fever

Unexplained weight loss

Bone pain
When to see a doctor
Contact your child's doctor if your child has any signs or symptoms that
worry you. Mention any changes in your child's behavior or habits.
Causes

In general, cancer begins with a genetic mutation that allows normal,

healthy cells to continue growing without responding to the signals to stop,
which normal cells do. Cancer cells grow and multiply out of control. The
accumulating abnormal cells form a mass (tumor).
Neuroblastoma begins in neuroblasts immature nerve cells that a fetus
makes as part of its development process.
As the fetus matures, neuroblasts eventually turn into nerve cells and fibers
and the cells that make up the adrenal glands. Most neuroblasts mature by
birth, though a small number of immature neuroblasts can be found in
newborns. In most cases, these neuroblasts mature or disappear. Others,
however, form a tumor a neuroblastoma.
It isn't clear what causes the initial genetic mutation that leads to
neuroblastoma.
Risk factors
Children with a family history of neuroblastoma may be more likely to
develop the disease. Yet, familial neuroblastoma is thought to comprise a
very small number of neuroblastoma cases. In most cases of
neuroblastoma, a cause is never identified.
Complications

Spread of the cancer (metastasis). Neuroblastoma may spread

(metastasize) to other parts of the body, such as the lymph nodes,
bone marrow, liver, skin and bones.

Spinal cord compression. Tumors may grow and press on the

spinal cord, causing spinal cord compression. Spinal cord
compression may cause pain and paralysis.

Signs and symptoms caused by tumor

secretions. Neuroblastoma cells may secrete certain chemicals that
irritate other normal tissues, causing signs and symptoms called
paraneoplastic syndromes. One paraneoplastic syndrome that occurs

rarely in people with neuroblastoma causes rapid eye movements and

difficulty with coordination. Another rare syndrome causes abdominal
swelling and diarrhea.
Tests and procedures used to diagnose neuroblastoma include:

Physical exam. Your child's doctor conducts a physical exam to

check out any signs and symptoms. The doctor will ask you questions
about your child's habits and behaviors.

Urine and blood tests. These may indicate the cause of any signs
and symptoms your child is experiencing. Urine tests may be used to
check for high levels of certain chemicals that result from the
neuroblastoma cells producing excess catecholamines.

Imaging tests. Imaging tests may reveal a mass that can indicate a
tumor. Imaging tests may include an X-ray, ultrasound, computerized
tomography (CT) scan, metaiodobenzylguanidine (MIBG) scan and
magnetic resonance imaging (MRI), among others.

Removing a sample of tissue for testing. If a mass is found, your

child's doctor may want to remove a sample of the tissue for
laboratory testing (biopsy). Specialized tests on the tissue sample can
reveal what types of cells are involved in the tumor and specific
genetic characteristics of the cancer cells. This information helps your
child's doctor devise an individualized treatment plan.

Removing a sample of bone marrow for testing. Your child may

also undergo bone marrow biopsy and bone marrow aspiration
procedures to see if neuroblastoma has spread to the bone marrow
the spongy material inside the largest bones where blood cells are
formed. In order to remove bone marrow for testing, a needle is
inserted into your child's hipbone or lower back to draw out the
marrow.
Staging
Once neuroblastoma is diagnosed, your child's doctor may order further
testing to determine the extent of the cancer and whether it has spread to
distant organs a process called staging. Imaging tests used to stage

cancer include X-rays, bone scans, and CT, MRI and MIBG scans, among
others.
Using the information from those procedures, your child's doctor assigns a
stage to the neuroblastoma. Stages of neuroblastoma include:

Stage I. Neuroblastoma at this stage is localized, meaning it's

confined to one area, and may be completely removed with surgery.
Lymph nodes connected to the tumor may have signs of cancer, but
other lymph nodes don't have cancer.

Stage IIA. Neuroblastoma at this stage is localized, but may not be

as easily removed through surgery.

Stage IIB. Neuroblastoma at this stage is localized and may or may

not be easily removed through surgery. Both the lymph nodes
connected to the tumor and the lymph nodes nearby contain cancer
cells.

Stage III. Neuroblastoma at this stage is considered advanced, and it

isn't possible to remove the tumor through surgery. The tumor may be
a larger size at this stage. Lymph nodes may or may not contain
cancer cells.

Stage IV. Neuroblastoma at this stage is considered advanced and

has spread (metastasized) to other parts of the body.

Stage IVS. This stage is a special category of neuroblastoma that

doesn't behave like other forms of neuroblastoma, though it isn't clear
why. Stage IVS neuroblastoma applies only to children younger than 1
year old. Stage IVS indicates that neuroblastoma has spread to
another part of the body most commonly the skin, liver or limited
bone marrow involvement. Despite the extent of neuroblastoma,
babies with this stage have a good chance of recovery.
Neuroblastoma at this stage sometimes goes away on its own and
often doesn't require any treatment.

Your child's doctor selects a treatment plan based on several factors

that affect your child's prognosis. Factors include your child's age, the

stage of the cancer, the type of cells involved in the cancer, and
whether there are any abnormalities in the chromosomes and genes
Your child's doctor uses this information to categorize the cancer as
low risk, intermediate risk or high risk. What treatment or combination
of treatments your child receives for neuroblastoma depends on the
risk category.

Surgery

Surgeons use scalpels and other surgical tools to remove cancer

cells. In children with low-risk neuroblastoma, surgery to remove the
tumor may be the only treatment needed.
Whether the tumor can be completely removed depends on its
location and its size. Tumors that are attached to nearby vital organs
such as the lungs or the spinal cord may be too risky to
remove.
In intermediate-risk and high-risk neuroblastoma, surgeons may try to
remove as much of the tumor as possible. Other treatments, such as
chemotherapy and radiation, may then be used to kill remaining
cancer cells.

Chemotherapy

Chemotherapy uses chemicals to destroy cancer cells.

Chemotherapy targets rapidly growing cells in the body, including
cancer cells. Unfortunately, chemotherapy also damages healthy
cells that grow quickly, such as cells in the hair follicles and in the
gastrointestinal system, which can cause side effects.
Children with intermediate-risk neuroblastoma often receive a
combination of chemotherapy drugs before surgery to improve the
chances that the entire tumor can be removed.
Children with high-risk neuroblastoma usually receive high doses of
chemotherapy drugs to shrink the tumor and to kill any cancer cells
that have spread elsewhere in the body. Chemotherapy is usually
used before surgery and before bone marrow stem cell transplant.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays, to

destroy cancer cells.
Children with low-risk or intermediate-risk neuroblastoma may
receive radiation therapy if surgery and chemotherapy haven't been
helpful. Children with high-risk neuroblastoma may receive radiation
therapy after chemotherapy and surgery, to prevent cancer from
recurring.
Radiation therapy primarily affects the area where it's aimed, but
some healthy cells may be damaged by the radiation. What side
effects your child experiences depends on where the radiation is
directed and how much radiation is administered.

Stem cell transplant

Children with high-risk neuroblastoma may receive a transplant using

their own blood stem cells (autologous stem cell transplant).
Before the stem cell transplant, your child undergoes a procedure
that filters and collects stem cells from his or her blood. The stems
cells are stored for later use. Then high doses of chemotherapy are
used to kill any remaining cancer cells in your child's body. Your
child's stem cells are then injected into your child's body, where they
can form new, healthy blood cells.

Immunotherapy

Immunotherapy uses drugs that work by signaling your body's

immune system to help fight cancer cells. Children with high-risk
neuroblastoma may receive immunotherapy drugs that stimulate the
immune system to kill the neuroblastoma cells.

Newer treatments

Doctors are studying a newer form of radiation therapy that may help
control high-risk neuroblastoma. The treatment uses a radioactive
form of the chemical metaiodobenzylguanidine (MIBG). When

injected in to the bloodstream, the MIBG travels to the neuroblastoma

cells and releases the radiation.
MIBG therapy is sometimes combined with chemotherapy or stem
cell transplant. After receiving an injection of the radioactive MIBG,
your child will need to stay in a special hospital room until the
radiation leaves his or her body in the urine. MIBG therapy usually
takes a few days.