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National Cancer Institute

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Neuroblastoma


Table of Contents

OVERVIEW OF PDQ
What is PDQ?
How to use PDQ
DESCRIPTION
STAGE EXPLANATION
Localized resectable
Localized unresectable
Regional
Disseminated
Stage IVS
Recurrent
TREATMENT OPTION OVERVIEW
Treatment by stage
LOCALIZED RESECTABLE NEUROBLASTOMA
LOCALIZED UNRESECTABLE NEUROBLASTOMA
REGIONAL NEUROBLASTOMA
DISSEMINATED NEUROBLASTOMA
STAGE IVS NEUROBLASTOMA
RECURRENT NEUROBLASTOMA
TO LEARN MORE

OVERVIEW OF PDQ


What is PDQ?

PDQ is a computer system that provides up-to-date information on cancer and its prevention, detection, treatment, and supportive care. PDQ is a service of the National Cancer Institute (NCI) for people with cancer and their families and for doctors, nurses, and other health care professionals.

To ensure that it remains current, the information in PDQ is reviewed and updated each month by experts in the fields of cancer treatment, prevention, screening, and supportive care. PDQ also provides information about research on new treatments (clinical trials), doctors who treat cancer, and hospitals with cancer programs. The treatment information in this summary is based on information in the PDQ summary for health professionals on this cancer.


How to use PDQ

Cancer in children and adolescents is rare. The majority of children with cancer are treated at cancer centers with special facilities to treat childhood cancers. There are organized groups of doctors and other health care professionals who work together by doing clinical trials to improve treatments for children with cancer.

PDQ can be used to learn more about current treatment of different kinds of cancer. You may find it helpful to discuss this information with your child's doctor, who knows your child and has the facts about your child's disease. PDQ can also provide the names of additional health care professionals and hospitals that specialize in treating children who have cancer.

Before your child begins treatment, you may want to consider entering your child in a clinical trial. PDQ can be used to learn more about the trials. A clinical trial is a research study that attempts to improve current treatments or find new treatments for people with cancer. Clinical trials are based on past studies and information discovered in the laboratory. Each trial answers specific scientific questions in order to find new and better ways to help people with cancer. During clinical trials, information is collected about new treatments, their risks, and how well they do or do not work. When a clinical trial shows that a new treatment is better than the treatment currently used as "standard" treatment, the new treatment may become the "standard" treatment. Children who are treated in clinical trials have the advantage of getting the best available therapy. In the United States, about two thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of current clinical trials are available on PDQ. In the United States, there are two major groups (called cooperative groups) that organize clinical trials for childhood cancers: the Childrens Cancer Group (CCG) and the Pediatric Oncology Group (POG). Doctors who belong to these groups or who take part in other clinical trials are listed in PDQ.

To learn more about cancer and how it is treated or to learn more about clinical trials for your child's kind of cancer, call the National Cancer Institute's Cancer Information Service. The number is 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615. The call is free and a trained information specialist will be available to answer your cancer-related questions.

PDQ is updated whenever there is new information. Check with the Cancer Information Service to be sure that you have the most up-to-date information.


DESCRIPTION

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 your child's cancer has spread), your child's age at diagnosis, the location of the tumor, and evaluation of the tumor cells under a microscope.


STAGE EXPLANATION

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.


Regional

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.


Disseminated

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 IVS).


Stage IVS

Stage IVS 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

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. If you want more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.


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. Listings of current clinical trials are available on PDQ or by calling the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER.


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. A clinical trial of new methods of treatment. Listings of current clinical trials are available on PDQ or by calling the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER.


STAGE IVS 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. Listings of current clinical trials are available on PDQ or by calling the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER.


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. Listings of current clinical trials are available on PDQ or by calling the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER.


TO LEARN MORE

TO LEARN MORE..... CALL 1-800-4-CANCER

To learn more about neuroblastoma, call the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800 332-8615. The call is toll-free and a trained information specialist can answer your questions.

The Cancer Information Service can also send you booklets. The following booklets on childhood cancer may be helpful to you:

Young People with Cancer: A Handbook for Parents
Talking with Your Child About Cancer
Managing Your Child's Eating Problems During Cancer Treatment
When Someone in Your Family Has Cancer

The following general booklets on topics related to cancer may also be helpful:

Taking Time: Support for People with Cancer and the People Who Care About
Them
What Are Clinical Trials All About?
Chemotherapy and You: A Guide to Self-Help During Treatment
Radiation Therapy and You: A Guide to Self-Help During Treatment
What You Need To Know About Cancer

There are many other places where material about cancer treatment and information about services are available. Check the hospital social service office for local and national agencies that help with finances, getting to and from treatment, care at home, and dealing with other problems.

Write to the National Cancer Institute at this address:

National Cancer Institute
Office of Cancer Communications
31 Center Drive, MSC 2580
Bethesda, MD 20892-2580

Date Last Modified: 09/1999


If you want to know more about cancer and how it is treated, or if you wish to know about clinical trials for your type of cancer, you can call the NCI's Cancer Information Service at 1-800-422-6237, toll free. A trained information specialist can talk with you and answer your questions.


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