General Information

What is neuroblastoma?

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 child's chance of recovery (prognosis) and choice of treatment depend on the stage of your child's cancer (how far the cancer has spread), your child's age at diagnosis, the location of the tumor, and evaluation of the tumor cells under a microscope.

Stage Information

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 child's 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:

Localized resectable
The cancer is confined to the site of origin, there is no evidence of spread, and the cancer can be surgically removed.

Localized unresectable
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
Stage 4S neuroblastoma is also called "special" 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.

Treatment Option Overview

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 patient's 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 patient's own marrow saved earlier and possibly treated with drugs to kill any cancer cells), allogeneic (marrow from a healthy "matched" donor, usually a brother or sister), or syngeneic (marrow from an identical twin).

Treatment By Stage

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.

Localized Resectable Neuroblastoma
Your child's treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery plus adjuvant chemotherapy.
3. Surgery plus radiation therapy.

Localized Unresectable Neuroblastoma
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.

Regional Neuroblastoma
Treatment depends on your child's age.
If your child is younger than 1 year of age, treatment may include the following:

1. Surgery to remove the cancer.
2. Chemotherapy.

If your child is older than 1 year of age, treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery followed by chemotherapy.
3. Chemotherapy with or without radiation therapy to reduce the tumor, followed by surgery.
4. Multi-drug chemotherapy.
5. Radiation therapy.
6. A clinical trial of new methods of treatment.

Disseminated Neuroblastoma
Your child's treatment may be one of the following:

1. Multi-drug chemotherapy with or without surgery and/or radiation therapy.
2. Chemotherapy followed by peripheral stem cell transplantation and 13-cis retinoic acid.
3. A clinical trial of new methods of treatment.

Stage 4S Neuroblastoma
Children with this special type of neuroblastoma may not require therapy. You may want to have your child take part in a clinical trial of new methods of treatment.

Recurrent Neuroblastoma
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. A clinical trial may be appropriate.

The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals.

The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation.

The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. Any use of the information will be at the risk of the user.

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