Neuroblastoma
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Neuroblastoma is a solid cancerous tumor that begins
in nerve tissue in the neck, chest, abdomen, or pelvis, but usually originates
in the abdomen in the tissues of the adrenal gland. By the time it is diagnosed,
the cancer usually has spread (metastasized), most commonly to the lymph nodes,
liver, lungs, bones, and bone marrow. Neuroblastoma is predominantly a tumor
of early childhood; two thirds of children with neuroblastoma are diagnosed
when they are younger than 5 years of age. It is often present at birth but
usually is not detected until later; in rare cases, neuroblastoma can be detected
before birth by fetal ultrasound.
The most common symptoms of neuroblastoma are the
result of pressure by the tumor or bone pain from cancer that has spread to
the bone. Protruding eyes and dark circles around the eyes are common and are
caused by cancer that has spread to the area behind the eye. Neuroblastomas
may compress the spinal cord, causing paralysis. Fever, anemia, and high blood
pressure are found occasionally. Rarely, children may have severe watery diarrhea,
uncoordinated or jerky muscle movements, or uncontrollable eye movement.
If your child has symptoms that may be caused by neuroblastoma,
his or her doctor will conduct a careful examination and order laboratory tests
and special x-rays. A computed tomographic (CT) scan, a diagnostic test that
uses computers and x-rays to create pictures of the body, may be performed.
A magnetic resonance imaging (MRI) scan, a diagnostic test similar to a CT
scan, but which uses magnetic waves instead of x-rays, may also be performed.
Often, removal of tissue from the tumor and/or bone
marrow is required to determine whether neuroblastoma exists. A small sample
of the tissue may be surgically removed and examined under a microscope. This
is called a biopsy. Sometimes a biopsy is done by making a small hole and using
a needle to extract a sample of the tissue.
Your childfs chance of recovery (prognosis) and choice
of treatment depend on the stage of your childfs cancer (how far the cancer
has spread), your childfs age at diagnosis, the location of the tumor, and
evaluation of the tumor cells under a microscope.
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Once neuroblastoma is found, more tests will be done
to find out if the cancer has spread from where it started to surrounding tissues
or other parts of the body. This is called staging. Your childfs doctor needs
to know the stage of the disease to plan treatment. Although there are several
staging systems currently available for neuroblastoma, for the purposes of
treatment the disease is categorized as follows:
The cancer is confined to the site of origin, there
is no evidence of spread, and the cancer can be surgically removed.
The cancer is confined to the site of origin, but
the cancer cannot be completely removed surgically.
The cancer has extended beyond the site of origin
to regional lymph nodes and/or surrounding organs or tissues, but has not spread
to distant parts of the body.
The cancer has spread from the site of origin to
distant lymph nodes, bone, liver, skin, bone marrow, and/or other organs (except
as defined for stage 4S).
Stage 4S neuroblastoma is also called gspecialh
neuroblastoma because it is treated differently. The cancer is localized, with
dissemination (spread) limited to liver, skin, and/or, to a very limited extent,
bone marrow.
Recurrent neuroblastoma means that the cancer has
come back (recurred) or continued to spread (progressed) after it has been
treated. It may come back in the original site or in another part of the body.
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There are treatments for all children with neuroblastoma.
Treatment options are related to age at diagnosis, tumor location, stage of
disease, regional lymph node involvement, and tumor biology. Four types of
treatment are used:
- Surgery (removing the tumor
in an operation)
- Radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer
cells and shrink tumors)
- Chemotherapy (using drugs to kill cancer cells and shrink tumors)
- Bone marrow transplantation (replacing the patientfs bone marrow with healthy
bone marrow)
More than one method of treatment may be used, depending
on the needs of the patient.
Surgery is used when possible to remove as much
of the cancer as possible. If the cancer cannot be removed, surgery may be
limited to a biopsy of the cancer.
Radiation therapy uses high-energy rays (radiation)
to damage or kill cancer cells and shrink tumors. Radiation usually comes from
a machine outside the body (external beam radiation therapy).
Chemotherapy is the use of drugs to kill cancer
cells and shrink tumors. Chemotherapy drugs may be taken by mouth or injected
into a vein (intravenous) or a muscle. Chemotherapy is called a systemic treatment
because the drug enters the bloodstream, travels through the body, and can
kill cancer cells throughout the body. Chemotherapy may be given after the
tumor has been surgically removed to kill any remaining cancer cells; this
is called adjuvant chemotherapy. Chemotherapy can also be given before surgery
to shrink the cancer so that it can be removed during surgery; this is called
neoadjuvant chemotherapy.
Bone marrow transplantation is a procedure in which
healthy bone marrow is given to replace bone marrow destroyed by treatment
with high doses of anticancer drugs or radiation. Transplantation may be autologous
(the patientfs own marrow saved earlier and possibly treated with drugs to
kill any cancer cells), allogeneic (marrow from a healthy gmatchedh donor,
usually a brother or sister), or syngeneic (marrow from an identical twin).
For the purposes of treatment presented here, neuroblastoma
is categorized as localized resected, localized unresected, regional, disseminated,
and special.
Your child may receive treatment that is considered
standard based on its effectiveness in a number of people in past studies,
or you may choose to enter your child in a clinical trial. Not all patients
are cured with standard therapy and some standard treatments may have more
side effects than are desired. For these reasons, clinical trials are designed
to test new treatments and to find better ways to treat people with cancer.
Clinical trials are ongoing in most parts of the country for most stages of
neuroblastoma.
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Your childfs treatment may be one of the following:
- Surgery to remove the cancer
- Surgery plus adjuvant chemotherapy
- Surgery plus radiation therapy.
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Initial treatment generally consists of surgical
removal of as much of the cancer as possible followed by
chemotherapy. A second surgery may be performed to remove any cancer
that remains, and radiation therapy may then be given.
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Treatment depends on your childfs age.
If your child is younger than 1 year of age, treatment
may include the following:
- Surgery to remove the cancer
- Chemotherapy
If your child is older than 1 year of age, treatment
may be one of the following:
- Surgery to remove the cancer
- Surgery followed by chemotherapy
- Chemotherapy with or without radiation
therapy to reduce the tumor, followed by surgery
- Multi-drug chemotherapy
- Radiation therapy
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Your childfs treatment may be one of the following:
- Multi-drug chemotherapy with or without
surgery and/or radiation therapy
- Chemotherapy followed by peripheral stem
cell transplantation and 13-cis retinoic acid.
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The selection of treatment of recurrent or progressive
neuroblastoma depends on the location and extent of the recurrence
or progression and on the previous therapy as well as individual
patient considerations.
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