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Neuroblastoma Treatment (PDQ®): Treatment

Last Modified : 2008-02-05

Neuroblastoma Treatment

General Information About Neuroblastoma

Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of 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 produce important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.

Neuroblastoma most often begins during early childhood, usually in children younger than 5 years. It sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth by fetal ultrasound.

By the time neuroblastoma is diagnosed, the cancer has usually metastasized (spread), most often to the lymph nodes, bones, bone marrow, liver, and skin.

(See the PDQ summary on Neuroblastoma Screening for more information.)

Possible signs of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.

The most common 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 symptoms may be caused by neuroblastoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:


Less common signs of neuroblastoma include the following:


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:


Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:


Prognosis and treatment decisions for neuroblastoma are also affected by tumor biology, which includes:


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.


Stages of Neuroblastoma

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, stage is one of the factors used to plan treatment. The following tests and procedures may be used to determine the stage:


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 stage 2A and 2B.


Stage 3

In stage 3, one of the following is true:


Stage 4

Stage 4 is divided into stage 4 and stage 4S.


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 3 risk groups: low risk, intermediate risk, and high risk.



Progressive/Recurrent Neuroblastoma

Progressive neuroblastoma is cancer that has progressed (continued to grow) during treatment. 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

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. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Children with neuroblastoma should have their treatment planned by a team of doctors with expertise in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric doctors who are experts in treating children with neuroblastoma and who specialize in certain areas of medicine. These may include the following specialists:


Children who are treated for neuroblastoma may be at higher risk for 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:


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.

Four types of standard treatment are used:
Surgery

Surgery is usually used to treat neuroblastoma. Depending on where the tumor is and whether it has spread, as much of the tumor as 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 and stage of the cancer being treated.

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 spinal column, 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 and stage of the cancer being treated.

The use of two or more anticancer drugs is called combination chemotherapy.

Watchful waiting

Watchful waiting is closely monitoring a patient's condition without giving any treatment until symptoms appear or change.

New types of treatment are being tested in clinical trials. These include the following:
Monoclonal antibody therapy

Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to deliver drugs, toxins, or radioactive material directly to cancer cells.

High-dose chemotherapy and radiation therapy with stem cell transplant

High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor 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.

Other drug therapy

13-cis retinoic acid is a vitamin-like drug that slows the cancer's ability to make more cancer cells and changes how these cells look and act.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.


Treatment Options for Neuroblastoma


Low-Risk Neuroblastoma

Treatment of low-risk neuroblastoma may include the following:


Information about ongoing clinical trials is available from the NCI Web site.


Intermediate-Risk Neuroblastoma

Treatment of intermediate-risk neuroblastoma may include the following:



High-Risk Neuroblastoma

Treatment of high-risk neuroblastoma may include the following:


This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.


Progressive/Recurrent Neuroblastoma

Patients First Treated for Low-Risk Neuroblastoma

Treatment for recurrent neuroblastoma that is found in one place in the body may include the following:


Treatment for recurrent neuroblastoma that has spread to other parts of the body may include the following:


Information about ongoing clinical trials is available from the NCI Web site.

Patients First Treated for Intermediate-Risk Neuroblastoma

For recurrent neuroblastoma that is found in one place in the body, treatment is usually surgery, with or without chemotherapy.

For recurrent neuroblastoma that has spread to other parts of the body, treatment is usually high-dose chemotherapy, stem cell transplant, and 13-cis retinoic acid.

Patients First Treated for High-Risk Neuroblastoma

Treatment of recurrent neuroblastoma in patients first treated for high-risk neuroblastoma may include the following:


This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with neuroblastoma.


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