Neuroblastoma

• Description
• What is neuroblastoma?
• Stage Explanation
• Localized resectable
• Localized unresectable
• Regional
• Disseminated
• Stage 4S
• Recurrent
• Treatment Option Overview
• Localized Resectable Neuroblastoma
• Localized Unresectable Neuroblastoma
• Regional Neuroblastoma
• Disseminated Neuroblastoma
• Stage 4S Neuroblastoma
• Recurrent Neuroblastoma
• To Learn More
• About PDQ

Description

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 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 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

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).

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:

• Surgery to remove the cancer
 
• Surgery plus adjuvant chemotherapy
 
• 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:

• 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
 
• 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:

• Multi-drug chemotherapy with or without surgery and/or radiation therapy
 
• Chemotherapy followed by peripheral stem cell transplantation and 13-cis retinoic acid.
 
• 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 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. 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

Call

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Web sites and Organizations

The NCI's Cancer.gov ¹ Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.

Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator ². These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

LiveHelp

The NCI's LiveHelp service, a program available on several of the Institute's Web sites, provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 10:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write

For more information from the NCI, please write to this address:

NCI Public Inquiries Office

Suite 3036A

6116 Executive Boulevard, MSC8322

Bethesda, MD 20892-8322

About PDQ

PDQ is a comprehensive cancer database available on Cancer.gov.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at Cancer.gov ¹, the NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."

Listings of clinical trials are included in PDQ and are available online at Cancer.gov ³. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on Cancer.gov ¹ or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.