Neuroblastoma
General Information
Signs and Symptoms
Stages
Treatment Overview
Neuroblastoma is a disease in which malignant (cancer) cells
form in certain types of nerve tissue. Neuroblastoma most often begins
in the adrenal glands, which are on top of the kidneys. It can also
form in nerve tissue in the neck, chest, abdomen, or spine.
Neuroblastoma most often occurs in children younger than 5
years of age. Sometimes it forms before birth and is found during a
routine pregnancy ultrasound. In children aged 6 months or younger, the
disease sometimes goes away without treatment.
Neuroblastoma is usually found when the tumor begins to grow
and cause signs or symptoms. By the time it is diagnosed, the cancer
has usually metastasized (spread to other parts of the body).
General Information About Neuroblastoma
Key Points
- Neuroblastoma is a disease in which malignant (cancer)
cells form in neuroblasts (immature nerve tissue) in the adrenal gland,
neck, chest, or spinal cord.
- Neuroblastoma is sometimes caused by a gene mutation
(change) passed from the parent to the child.
- Signs and symptoms of neuroblastoma include bone pain and a
lump in the abdomen, neck, or chest.
- Tests that examine many different body tissues and fluids
are used to detect (find) and diagnose neuroblastoma.
- A biopsy is done to diagnose neuroblastoma.
- Certain factors affect prognosis (chance of recovery) and
treatment options.
Neuroblastoma
is a disease in which malignant (cancer) cells form in neuroblasts
(immature nerve tissue) in the adrenal gland, neck, chest, or spinal
cord.
Neuroblastoma often begins
in the nerve tissue of the adrenal glands. There are
two adrenal glands, one on top of each kidney
in the back of the upper abdomen.
The adrenal glands make important hormones
that help control heart rate, blood
pressure, blood
sugar, and the way the body reacts to stress. Neuroblastoma may
also begin in nerve tissue in the neck, chest, abdomen or pelvis.
Neuroblastoma may be found
in the adrenal glands and paraspinal nerve tissue from the neck to the
pelvis.
Neuroblastoma most often
begins in infancy and may be diagnosed
in the first month of life. It is found when the tumor begins to grow and
cause signs or symptoms. Sometimes it forms
before birth and is found during a fetal
ultrasound.
By the time neuroblastoma
is diagnosed, the cancer has usually metastasized (spread).
Neuroblastoma spreads most often to the lymph
nodes, bones, bone
marrow, and liver.
In infants, it also spreads to the skin.
Neuroblastoma
is sometimes caused by a gene mutation (change) passed from the parent
to the child.
Gene mutations that increase the
risk of neuroblastoma are sometimes inherited
(passed from the parent to the child). In children with a gene
mutation, neuroblastoma usually occurs at a younger age and more than
one tumor may form in the adrenal glands.
Signs
and symptoms of neuroblastoma include bone pain and a lump in the
abdomen, neck, or chest.
The most common signs and
symptoms of neuroblastoma are caused by the tumor pressing on nearby
tissues as it grows or by cancer spreading to the bone. These and other
signs and symptoms may be caused by neuroblastoma or by other conditions.
Check with your child's
doctor if your child has any of the following:
- Lump in the abdomen, neck, or chest.
- Bulging eyes.
- Dark circles around the eyes ("black eyes").
- Bone pain.
- Swollen stomach
and trouble breathing (in infants).
- Painless, bluish lumps under the skin (in infants).
- Weakness or paralysis
(loss of ability to move a body part).
Less common signs and
symptoms of neuroblastoma include the following:
- Fever.
- Shortness of breath.
- Feeling tired.
- Easy bruising or bleeding.
- Petechiae
(flat, pinpoint spots under the skin caused by bleeding).
- High blood pressure.
- Severe watery diarrhea.
- Horner syndrome
(droopy eyelid, smaller pupil,
and less sweating on one side of the face).
- Jerky muscle movements.
- Uncontrolled eye movements.
Tests
that examine many different body tissues and fluids are used to detect
(find) and diagnose neuroblastoma.
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
person'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.
- Urine
catecholamine
studies: A procedure in which a urine sample is checked
to measure the amount of certain substances, vanillylmandelic acid
(VMA) and homovanillic acid (HVA), that are made when catecholamines
break down and are released into the urine. A higher than normal amount
of VMA or HVA can be a sign of neuroblastoma.
- Blood
chemistry studies : A procedure in which a
blood sample is
checked to measure the amounts of certain substances released into the
blood by organs and
tissues in the body. An unusual (higher or lower than normal) amount of
a substance can be a sign of disease.
- X-ray
: An x-ray is a type of energy beam that can go
through the body and onto film, making a picture of areas inside the
body.
- 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.
Computed tomography
(CT) scan of the abdomen. The child lies on a table that slides through
the CT scanner, which takes x-ray pictures of the inside of the abdomen.
- MRI
(magnetic resonance imaging) with gadolinium
: A procedure that uses a magnet, radio waves, and a computer
to make a series of detailed pictures of areas inside the body. 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).
Magnetic resonance
imaging (MRI) of the abdomen. The child lies on a table that slides
into the MRI scanner, which takes pictures of the inside of the body.
The pad on the child's abdomen helps make the pictures clearer.
- mIBG
(metaiodobenzylguanidine) scan : A
procedure used to find neuroendocrine
tumors, such as neuroblastoma. A very small amount of a
substance called radioactive
mIBG is injected
into a vein and
travels through the bloodstream. Neuroendocrine tumor cells take up the
radioactive mIBG and are detected by a scanner.
Scans may be taken over 1-3 days. An iodine
solution may be given before or during the test to keep the thyroid gland from absorbing
too much of the mIBG. This test is also used to find out how well the
tumor is responding
to treatment. mIBG is used in high doses
to treat neuroblastoma.
- 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 marrow aspiration
and biopsy. After a small area of skin is numbed, a bone marrow needle
is inserted into the child's hip bone. Samples of blood, bone, and bone
marrow are removed for examination under a microscope.
- Ultrasound exam: A procedure
in which high-energy sound waves (ultrasound) are bounced off internal
tissues or organs and make echoes. The echoes form a picture of body
tissues called a sonogram.
The picture can be printed to be looked at later. An ultrasound exam is
not done if a CT/MRI has been done.
Abdominal ultrasound.
An ultrasound transducer connected to a computer is pressed against the
skin of the abdomen. The transducer bounces sound waves off internal
organs and tissues to make echoes that form a sonogram (computer
picture).
A
biopsy is done to diagnose neuroblastoma.
Cells and tissues are
removed during a biopsy
so they can be viewed under a microscope by a pathologist to check for
signs of cancer. The way the biopsy is done depends on where the tumor
is in the body. Sometimes the whole tumor is removed at the same time
the biopsy is done.
The following tests may
be done on the tissue that is removed:
- 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.
- 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.
- Immunohistochemistry
: A test that uses antibodies
to check for certain antigens
in a sample of tissue. The antibody is usually linked to a radioactive
substance or a dye that causes the tissue to light up under a
microscope. This type of test may be used to tell the difference
between different types of cancer.
- MYCN
amplification study : A laboratory study
in which tumor or bone marrow cells are checked for the level of MYCN.
MYCN is important for cell growth. A higher
level of MYCN (more than 10 copies of the gene)
is called MYCN amplification. Neuroblastoma with MYCN
amplification is more likely to spread in the body and less likely to
respond to treatment.
Children up to 6 months
old may not need a biopsy or surgery
to remove the tumor because the tumor may disappear without treatment.
Certain
factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment
options depend on the following:
- Age of the child at the time of diagnosis.
- The child's risk
group.
- Whether there are certain changes in the genes.
- Where in the body the tumor started.
- Tumor histology
(the shape, function, and structure of the tumor cells).
- Whether there is cancer in the lymph nodes on the same side
of the body as the primary cancer or whether there is cancer in the
lymph nodes on the opposite side of the body.
- How the tumor responds to treatment.
- How much time passed between diagnosis and when the cancer recurred (for recurrent cancer).
Prognosis and treatment
options for neuroblastoma are also affected by tumor biology, which
includes:
- The patterns of the tumor cells.
- How different the tumor cells are from normal cells.
- How fast the tumor cells are growing.
- Whether the tumor shows MYCN
amplification.
- Whether the tumor has changes in the ALK gene.
The tumor biology is
said to be favorable or unfavorable, depending on these factors. A
favorable tumor biology means there is a better chance of recovery.
In some children up to 6
months old, neuroblastoma may disappear without treatment. This is
called spontaneous regression. The child is closely watched for signs
or symptoms of neuroblastoma. If signs or symptoms occur, treatment may
be needed.
Stages of Neuroblastoma
Key Points
- After neuroblastoma has been diagnosed, tests are done to
find out if cancer has spread from where it started to other parts of
the body.
- There are three ways that cancer spreads in the body.
- Cancer may spread from where it began to other parts of the
body.
- The following stages are used for neuroblastoma:
- Stage 1
- Stage 2
- Stage 3
- Stage 4
- Treatment of neuroblastoma is based on risk groups.
After
neuroblastoma has been diagnosed, tests are done to find out if cancer
has spread from where it started to other parts of the body.
The process used to find
out the extent or spread of cancer
is called staging.
The information gathered from the staging process helps determine the stage of the disease. For neuroblastoma, the stage of
disease affects whether the cancer is low risk, intermediate risk, or
high risk. It also affects the treatment
plan. The results of some tests and procedures used to diagnose neuroblastoma may
be used for staging. See the General Information section for a
description of these tests and procedures.
The following tests and
procedures also may be used to determine the stage:
- Lymph
node biopsy
: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to
look for cancer cells.
One of the following types of biopsies may be done:
- Excisional
biopsy : The removal of an entire lymph
node.
- Incisional
biopsy : The removal of part of a lymph
node.
- Core
biopsy : The removal of tissue from a
lymph node using a wide needle.
- Fine-needle
aspiration (FNA) biopsy : The removal of
tissue or fluid
from a lymph node using a thin needle.
- X-ray
of the bone: An x-ray is a type of energy beam that can
go through the body and onto film, making a picture of areas inside the
body.
- 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 with cancer and is detected by a scanner.
There
are three ways that cancer spreads in the body.
Cancer can spread
through tissue, the
lymph system, and
the blood:
- Tissue. The cancer spreads from where it began by growing
into nearby areas.
- Lymph system. The cancer spreads from where it began by
getting into the lymph system. The cancer travels through the lymph vessels to other parts
of the body.
- Blood. The cancer spreads from where it began by getting
into the blood. The cancer travels through the blood vessels to other parts
of the body.
Cancer
may spread from where it began to other parts of the body.
When cancer spreads
to another part of the body, it is called metastasis.
Cancer cells break
away from where they began (the primary
tumor) and travel through the lymph system or blood.
- Lymph system. The cancer gets into the lymph system,
travels through the lymph vessels, and forms a tumor (metastatic tumor) in another
part of the body.
- Blood. The cancer gets into the blood, travels through the
blood vessels, and forms a tumor (metastatic tumor) in another part of
the body.
The metastatic tumor is
the same type of cancer as the primary tumor. For example, if
neuroblastoma spreads to the liver,
the cancer cells in the liver are actually neuroblastoma cells. The
disease is metastatic neuroblastoma, not liver cancer.
The
following stages are used for neuroblastoma:
Stage
1
In stage 1, the tumor is in only one area
and all of the tumor that can be seen is completely removed during surgery.
Stage
2
Stage 2 is divided into
stages 2A and 2B.
- Stage 2A:
The tumor is in
only one area and all of the tumor that can be seen cannot be
completely removed during surgery.
- Stage 2B:
The tumor is in
only one area and all of the tumor that can be seen may
be completely removed during surgery.
Cancer cells are found in the lymph nodes near the tumor.
Stage
3
In stage 3, one of the
following is true:
- the tumor
cannot be completely removed during surgery
and has spread from one side of the body to the other side and may also
have spread to nearby lymph nodes;
or
- the tumor is in only one area, on one side of the body, but
has spread to lymph nodes on the other side of the body; or
- the tumor is in the middle of the body and has spread to tissues or lymph nodes on
both sides of the body, and the tumor cannot be removed by surgery.
Stage
4
Stage 4 is divided into
stages 4 and 4S.
- In stage 4,
the tumor has
spread to distant lymph nodes
or other parts of the body.
- In stage 4S:
- the child is younger than 12 months; and
- the cancer
has spread to the skin, liver,
and/or bone marrow;
and
- the tumor
is in only one area and all of the tumor that can be seen may
be completely removed during surgery;
and/or
- cancer cells
may be found in the lymph nodes
near the tumor.
Treatment
of neuroblastoma is based on risk groups.
For many types of
cancer, stages are used to plan treatment. For neuroblastoma, treatment
depends on risk groups.
The stage of neuroblastoma is one factor used to determine risk group.
Other factors are the age of the child, tumor histology, and tumor biology.
There are three risk
groups: low risk, intermediate risk, and high risk.
- Low-risk and intermediate-risk neuroblastoma have a good
chance of being cured.
- High-risk neuroblastoma may be hard to cure.
Recurrent Neuroblastoma
Recurrent neuroblastoma is cancer that has recurred (come back) after
it has been treated. The cancer may come back in the same place or in
other parts of the body.
Treatment Option Overview
Key Points
- There are different types of treatment for patients with
neuroblastoma.
- Children with neuroblastoma should have their treatment
planned by a team of doctors who are experts in treating childhood
cancer, especially neuroblastoma.
- Children who are treated for neuroblastoma may have late
effects, including an increased risk of second cancers.
- Seven types of standard treatment are used:
- Observation
- Surgery
- Radiation therapy
- Iodine 131-mIBG therapy
- Chemotherapy
- High-dose chemotherapy and radiation therapy with stem
cell rescue
- Targeted therapy
- New types of treatment are being tested in clinical trials.
- Immunotherapy
- Other drug therapy
- Patients may want to think about taking part in a clinical
trial.
- Patients can enter clinical trials before, during, or after
starting their cancer treatment.
- Follow-up tests may be needed.
There
are different types of treatment for patients with neuroblastoma.
Different types of
treatment are available for patients with neuroblastoma.
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
with neuroblastoma should have their treatment planned by a team of
doctors who are experts in treating childhood cancer, especially
neuroblastoma.
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 neuroblastoma and who specialize
in certain areas of medicine.
These may include the following specialists:
- Pediatric surgeon.
- Pediatric radiation
oncologist..
- Endocrinologist.
- Neurologist.
- Pediatric neuropathologist..
- Neuroradiologist.
- Pediatrician.
- Pediatric nurse
specialist.
- Social worker.
- Child life
professional.
- Psychologist.
Children
who are treated for neuroblastoma may have late effects, including an
increased risk of second cancers.
Some cancer treatments
cause side effects
that continue or appear years after cancer treatment has ended. These
are called late effects.
Late effects of cancer treatment may include:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers
(new types of cancer).
Some late effects may
be treated or controlled. It is important that parents of children who
are treated for neuroblastoma talk with their doctors about the
possible late effects caused by some treatments. See the PDQ summary on Late Effects
of Treatment for Childhood Cancer for more information.
Seven
types of standard treatment are used:
Observation
Observation is closely monitoring a patient's condition without giving any
treatment until signs
or symptoms appear
or change.
Surgery
Surgery is used to treat
neuroblastoma unless it has spread to other parts of the body.
Depending on where the tumor
is, as much of the tumor as is safely possible will be removed. If the
tumor cannot be removed, a biopsy
may be done instead.
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.
The way the radiation
therapy is given depends on the type of cancer being treated and the
child's risk group. External radiation therapy is used to treat
neuroblastoma.
Iodine
131-mIBG therapy
Iodine 131-mIBG therapy is a treatment with radioactive iodine. The
radioactive iodine is given through an intravenous
(IV) line and enters the bloodstream which carries radiation directly
to tumor cells. Radioactive iodine collects in neuroblastoma cells and
kills them with the radiation that is given off. Iodine 131-mIBG
therapy is sometimes used to treat high-risk neuroblastoma that comes
back after initial treatment.
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 cerebrospinal
fluid, an organ,
or a body cavity
such as the abdomen,
the drugs mainly affect cancer cells in those areas (regional chemotherapy). The
way the chemotherapy is given depends on the type of cancer being
treated and the child's risk
group.
The use of two or more anticancer drugs is called combination chemotherapy.
See Drugs Approved for
Neuroblastoma for more information.
High-dose
chemotherapy and radiation therapy with stem cell rescue
High-dose chemotherapy and
radiation therapy with stem cell
rescue is a way of giving high doses
of chemotherapy and radiation therapy and replacing blood -forming cells
destroyed by cancer treatment for high-risk neuroblastoma. Stem cells (immature blood
cells) are removed from the blood or bone
marrow of the patient and are frozen and stored. After
chemotherapy and radiation therapy are 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.
Maintenance therapy is given
after high-dose chemotherapy and radiation therapy with stem cell
rescue to kill any cancer cells that may regrow and cause the disease
to come back. Maintenance therapy is given for 6 months and includes
the following treatments:
- Isotretinoin:
A vitamin -like
drug that slows the cancer's ability to make more cancer cells and
changes how these cells look and act. This drug is taken by mouth.
- Dinutuximab:
A type of monoclonal antibody
therapy that uses an antibody
made in the laboratory from a single type of immune system cell.
Dinutuximab identifies and attaches to a substance, called GD2, on the
surface of neuroblastoma cells. Once dinutuximab attaches to the GD2, a
signal is sent to the immune system that a foreign
substance has been found and needs to be killed. Then the body's immune
system kills the neuroblastoma cell. Dinutuximab is given by infusion.
It is a type of targeted therapy.
- Granulocyte-macrophage
colony-stimulating factor (GM-CSF): A cytokine that helps make
more immune system cells, especially granulocytes
and macrophages (white blood cells), which
can attack and kill cancer cells.
- Interleukin-2
(IL-2): A type of immunotherapy
that boosts the growth and activity of many immune cells, especially lymphocytes (a type of white
blood cell). Lymphocytes can attack and kill cancer cells.
See Drugs Approved for
Neuroblastoma for more information.
Targeted
therapy
Targeted therapy is a
type of treatment that uses drugs or other substances to identify and
attack cancer cells with less harm to normal cells. There are different
types of targeted therapy:
- Tyrosine kinase
inhibitors are small-molecule
drugs that go through the cell membrane
and work inside cancer cells to block signals that cancer cells need to
grow and divide. Crizotinib
is used to treat neuroblastoma that has come back after treatment.
- Antibody-drug
conjugates are made up of a monoclonal
antibody attached to a drug. The monoclonal antibody binds
to specific proteins
or receptors found
on certain cells, including cancer cells. The drug enters these cells
and kills them without harming other cells. Lorvotuzumab mertansine is
an antibody-drug conjugate being studied to treat neuroblastoma that
has come back after treatment.
New
types of treatment are being tested in clinical trials.
This summary 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 website.
Immunotherapy
Immunotherapy is a treatment
that uses the patient's immune
system to fight cancer. Substances made by the body or
made in a laboratory are used to boost, direct, or restore the body's
natural defenses against cancer. This type of cancer treatment is also
called biotherapy or biological therapy.
Vaccine therapy uses a
substance to stimulate the immune system to destroy a tumor. Vaccine
therapy is being studied to treat neuroblastoma that has come back
after treatment.
Other
drug therapy
Lenalidomide is a type of angiogenesis inhibitor. It
prevents the growth of new blood
vessels that are needed by a tumor to grow.
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.
Clinical trials are
taking place in many parts of the country. See the Treatment Options
section that follows for links to current treatment clinical trials.
These have been retrieved from NCI's
listing of clinical trials.
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.
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 cancer has recurred
(come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Neuroblastoma
Low-Risk Neuroblastoma
Treatment of low-risk neuroblastoma may include
the following:
- Surgery
followed by observation.
- Chemotherapy
with or without surgery, for children with symptoms,
children whose tumor
has continued to grow and cannot be removed by surgery, or children
with unfavorable tumor biology.
- Observation alone for infants younger than 6 months who
have small adrenal
tumors.
- Observation alone for infants who do not have signs or symptoms of neuroblastoma.
- Radiation therapy
to treat tumors that are causing serious problems and do not respond quickly to
chemotherapy or surgery.
- A clinical trial
of treatment based on the tumor's response
to treatment and tumor biology.
Intermediate-Risk Neuroblastoma
Treatment of
intermediate-risk neuroblastoma
may include the following:
- Chemotherapy
for children with symptoms
or to shrink a tumor
that cannot be removed by surgery.
Surgery may be done after chemotherapy.
- Surgery alone for infants.
- Observation
alone for certain infants.
- Radiation therapy
to treat tumors that are causing serious problems and do not respond quickly to
chemotherapy or surgery.
- Radiation therapy for tumors that do not respond to other
treatment.
- A clinical trial
of treatment based on the tumor's response
to treatment and tumor biology.
High-Risk Neuroblastoma
Treatment of high-risk neuroblastoma may include
the following:
- A regimen
of combination chemotherapy,
surgery, stem cell rescue, radiation therapy, and monoclonal antibody therapy (dinutuximab) with interleukin-2 (IL-2), granulocyte-macrophage colony-stimulating
factor (GM-CSF), and isotretinoin.
Stage 4S Neuroblastoma
There is no standard treatment for stage 4S neuroblastoma but
treatment options include the following:
- Observation
with supportive care
for certain children who have favorable tumor biology and do not have signs or symptoms.
- Chemotherapy,
for children who have signs or symptoms of neuroblastoma
or unfavorable tumor biology, or for very young infants.
- A clinical trial
of treatment based on the tumor's response
to treatment and tumor biology.
Recurrent Neuroblastoma
Patients
First Treated for Low-Risk Neuroblastoma
Treatment for recurrent neuroblastoma that is found
only in the area where the cancer first formed may include the
following:
- Surgery
followed by observation
or chemotherapy.
- Chemotherapy that may be followed by surgery.
Treatment for recurrent
neuroblastoma that has spread to other parts of the body may include
the following:
- Observation.
- Chemotherapy.
- Surgery followed by chemotherapy.
- Treatment as for newly diagnosed high-risk neuroblastoma
for children older than 1 year.
Patients
First Treated for Intermediate-Risk Neuroblastoma
Treatment for recurrent neuroblastoma that is found
only in the area where the cancer first formed may include the
following:
- Surgery
that may be followed by chemotherapy.
- Treatment as for newly diagnosed high-risk neuroblastoma
for neuroblastoma that has spread to other parts of the body.
Recurrent neuroblastoma
that has spread to other parts of the body is treated the same way as
newly diagnosed high-risk neuroblastoma.
Patients
First Treated for High-Risk Neuroblastoma
Treatment for recurrent neuroblastoma may include
the following:
- Combination
chemotherapy.
- Iodine 131-mIBG
therapy to relieve symptoms
and improve quality of life.
It may be given alone or in combination
with other therapy, or before stem
cell rescue.
- A second course of high-dose
chemotherapy and stem cell rescue.
- Tyrosine kinase
inhibitor therapy (crizotinib)
for patients with changes in the ALK gene.
Because there is no standard treatment for
recurrent neuroblastoma in patients first treated for high-risk
neuroblastoma, patients may want to consider a clinical trial. For
information about clinical trials, please see the NCI website.
Patients
with Recurrent CNS Neuroblastoma
Treatment for neuroblastoma that recurs (comes back) in the central nervous system (CNS;
brain and spinal cord)
may include the following:
- Surgery
to remove the tumor
in the CNS followed by radiation
therapy.
- A clinical trial
of a new therapy.
Treatments
Being Studied for Progressive/Recurrent Neuroblastoma
Some of the treatments
being studied in clinical trials
for neuroblastoma
that recurs (comes
back) or progresses
(grows, spreads, or does not respond
to treatment) include the following:
- Combination
chemotherapy and monoclonal
antibody therapy
(dinutuximab).
- Lenalidomide
and monoclonal antibody therapy (dinutuximab) with or without isotretinoin.
- Iodine 131-mIBG
given alone or with other anticancer drugs.
- Immunotherapy
(vaccine therapy).
- Tyrosine kinase
inhibitor (crizotinib)
and combination chemotherapy.
- Targeted therapy
with an antibody-drug conjugate
( lorvotuzumab mertansine).
-NIH
