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Neuroblastoma

Neuroblastoma is a solid cancerous tumor that most commonly originates in the abdomen in the adrenal gland, but can also start in nerve tissue in the neck, chest, abdomen, or pelvis. At diagnosis, in approximately 70% of patients, the cancer 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 may be present at birth but usually is not detected until later; in rare cases, neuroblastoma is detected before birth through fetal therapy.

The most common symptoms of neuroblastoma are the result of pressure by the tumor mass, or pain from cancer that has spread to the bone. Protruding eyes and dark circles around the eyes are common and caused by cancer that has spread to the area behind the eye. Neuroblastomas may compress the spinal cord, causing paralysis. There can be distention of the abdomen due to a large tumor mass. 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 most likely refer you to a pediatric oncologist. The oncologist 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. A bone scan will be needed as well.

Removal of a sample of tissue from the tumor and/or a bone marrow aspiration and biopsy are required to determine whether the diagnosis is neuroblastoma, and whether it has spread to the bone marrow. A small sample of tissue will from the most accessible part of the tumor will be surgically removed by an oncologic surgeon and examined under a microscope. This is called a biopsy. Sometimes a biopsy is done by making a small hole in the skin 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 primary tumor, and evaluation of the tumor cells under a microscope. It also depends on some tests that are done on the tumor looking at genetic abnormalities unique to neuroblastoma.

Staging of neuroblastoma

Once neuroblastoma is diagnosed, 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 stage of disease is categorized as follows:

Localized resectable

The cancer is confined to the site of origin, there is no obvious evidence of spread, and the cancer can be surgically removed. These children have a greater than 90% 5-year survival.

Localized Unresectable

The cancer is confined to the site of origin but the cancer cannot be completely removed surgically. These children have a 75-90% long-term survival, depending upon age, tumor biology and therapy.

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. Children younger than one year of age have approximately 80% chance of cure, while older children have a cure rate of 50% - 70% with aggressive treatment.

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, see below). Children younger than one year of age have a 50-80% 5 year survival rate, while children older than one year have a 10-40% survival rate.

Stage IVS

Stage IVS neuroblastoma is also called "special" neuroblastoma. It is found in children less than one year of age and treated differently than other neuroblastoma. The cancer is localized, with dissemination (spread) limited to liver, skin, and/or, minimal bone marrow involvement. Survival ranges from 80-100% in these infants.

Stage, age and tumor biology (histology) are used to assign patients to a risk group that determines treatment.

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. The prognosis in these children depends upon many factors but is usually poor despite intensive therapies.

Treatment option overview

There are treatments available 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:

  1. Surgery (removing the tumor in an operation).
  2. Radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells and shrink tumors).
  3. Chemotherapy (using drugs to kill cancer cells and shrink tumors).
  4. Bone marrow/stem cell transplantation (replacing the patient's bone marrow with healthy bone marrow or peripheral blood stem cells).
Surgery

More than one method of treatment is usually used. Surgery is used when possible to remove as much of the cancer as possible. If the cancer cannot be removed, surgery is limited to an initial biopsy of the cancer. Then after chemotherapy has shrunk the tumor as much as possible, another surgery takes place to get rid of bulk disease.

Radiation therapy

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

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 will be given even if the tumor has been surgically removed to kill any remaining cancer cells; this is called adjuvant chemotherapy. Chemotherapy for neuroblastoma is more commonly given after a biopsy but before definitive surgery, to shrink the cancer so that it can be more easily removed during definitive surgery. This is called neoadjuvant chemotherapy.

Bone marrow transplantation

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 or stem cells obtained earlier and stored after being 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 patients in past clinical trials, or you may choose to enter your child in a clinical trial. Clinical trials are designed to test potentially better treatments against those treatments currently considered standard of care in hope of finding better ways to treat people with cancer. Clinical trials are ongoing in most parts of the country for most stages of neuroblastoma. The good news is that Children's Oncology Group clinical trials are closely overseen by a data monitoring committee, so that in real time, if one treatment is found to be better than another, the treating physicians are notified immediately, and your child's treatment will be changed right away if necessary.

Localized resectable neuroblastoma

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

  1. Surgery to remove the cancer.
  2. Surgery plus adjuvant chemotherapy in cases of life or organ threatening situations.
  3. Surgery plus radiation therapy in-patients with symptomatic life or organ threatening situations in which the tumor does not respond quickly enough to chemotherapy.
Localized unresectable neuroblastoma

Initial treatment in low risk patients generally consists of surgical biopsy or removal of as much of the cancer as possible, followed by chemotherapy if less than 50% of the tumor is removed. If more than 50% of the tumor can be removed, systematic observation may be utilized. A second surgery may be performed to remove any cancer that remains after chemotherapy. Radiation therapy may then be given. In patients classified as high risk, chemotherapy is given prior to attempted removal of the primary tumor, followed by high dose chemotherapy and/or radiation therapy, followed by autologous bone marrow or stem cell transplantation. Radiation therapy to residual tumor not able to be surgically removed will also occur. After recovery from transplantation, oral medications will 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, followed by chemotherapy if less than 50% of the tumor is surgically removed.
  2. Chemotherapy is given for life or organ threatening situations.

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

  1. Surgery to remove the cancer.
  2. Surgery followed by chemotherapy.
  3. Chemotherapy with or without radiation to reduce the tumor, followed by surgery and/or stem cell transplantation.
  4. Multi-drug chemotherapy.
  5. Radiation therapy.
Disseminated neuroblastoma

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

  1. Multi-drug chemotherapy with or without surgery and/or radiation therapy, followed by cellular differentiation therapy.
  2. Multidrug chemotherapy with or without surgery and/or radiation therapy, followed by stem cell reconstitution and cellular differentiation therapy.
Stage IVS neuroblastoma

Children with this special type of neuroblastoma may not require therapy, but this is dependent upon risk category. 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, the extent of the recurrence or progression, the previous therapy as well as individual patient considerations such as risk category. A clinical trial may be appropriate.

Cleveland Clinic Children's

Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Should you have more questions about the child or young adult in your life with cancer, please contact Cleveland Clinic Cancer Answer line at 216.444.3024 or 800.223.2273, extension 4-3024. Your question will be forwarded to the Chair of the Pediatric Oncology Department.

We wish you and your child well.

For other websites regarding childhood cancer:

Source: National Institutes of Health, National Cancer Institute, Children's Oncology Group

© 1995-2009 The Cleveland Clinic Foundation. All rights reserved.

This information is provided by the Cleveland Clinic Health System and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. Rev. 1/03


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