On this page:

• About Neuroblastoma
• Symptoms
• Diagnosis
• Stages
• Treatment
• Treatment by Stage
• Clinical Trials

About Neuroblastoma

Neuroblastoma is a cancer of the nerve tissues that is most often found in children under 5 years old. In 70 percent of cases, neuroblastoma starts in the adrenal gland, located in the abdomen. Other growth sites it favors in the early stages are nerve tissue of the neck, chest, abdomen, pelvis and near the spine. By the time it is diagnosed it has usually spread (metastasized) to the lymph nodes, liver, lungs, bones or bone marrow. Neuroblastoma is sometimes present at birth, but not detected. In rare cases, however, it can be identified before birth by ultrasound testing of the fetus.

• Neuroblastoma is the most common type of cancer in infants.
• The number of new cases of neuroblastoma is greatest among children under 1 year of age and decreases rapidly with age.
• Males are affected slightly more commonly than females.
• Most cases of neuroblastoma are diagnosed before 6 months of age.
• Unlike most cancers, neuroblastoma sometimes disappears — regresses — by itself. Researchers don't know why this is, and can't predict when it will happen.
• In a large percentage of patients, a bone marrow transplant is a successful treatment option.

Back to top

Symptoms

Common symptoms include:

• Protruding eyes and dark circles around the eyes.
• Pale color.
• In rare cases, watery diarrhea, uncoordinated or jerky muscle movements and uncontrollable eye movement.
• Enlarged abdomen

Back to top

Diagnosis

If symptoms suggest a child has neuroblastoma, a physician will conduct further tests to discover the site and extent of the cancer. Among these tests are:

• A CT scan to determine if there is a lump, or mass, in the abdomen or chest
• MIBG and/or bone scan to determine all sites of disease, including bones
• Urine test for chemical markers of the disease.
• Bone marrow biopsy: If results of the blood test are not normal, bone marrow may be examined. It is extracted through a thin needle inserted into the hip, after first numbing the area with an injection. The marrow will be examined under the microscope to examine abnormal cells.

A child's chances of recovery and the kind of treatment prescribed depend on four clinical variables:

• How far the cancer has spread — referred to as its "stage" (see below)
• Child's age at diagnosis
• Location of tumor
• Biological characteristics of the tumor when examined microscopically

Back to top

Stages

Once neuroblastoma is found, tests will be done to find out the extent to which it has spread from its point of origin to surrounding tissues, or other parts of the body. This is called "staging" the cancer. The patient's doctor needs to know the stage of the cancer to recommend the most effective treatments. The six stages of neuroblastoma are:

1. Localized resectable: cancer has not spread and as a result, can be surgically removed, or "resected."
2. Localized unresectable: cancer has not spread — but it cannot be completely removed by surgery.
3. Regional: cancer has spread beyond the site of origin to nearby lymph nodes and/or nearby tissue, but it has not spread to distant parts of the body.
4. Disseminated (Stage IV): cancer has spread beyond the site of origin to lymph nodes, bone, liver, skin, bone marrow and/or other organs.
5. Stage IVS: also referred to as "special" neuroblastoma because it is treated differently. The cancer is localized, with limited spread to liver, skin or minimally in the bone marrow, and the child is less than 1 year of age.
6. Recurrent: neuroblastoma that has come back (recurred) or continued to spread even after treatment. It may come back to the original site, or in another part of the body.

Back to top

Treatment

All children with neuroblastoma can be treated. Four types of treatments are used, either singly, or in combination, depending on the location, stage and biological characteristics of the cancer cells.

• Surgery to remove the cancer. This is used whenever possible.
• Radiation therapy to kill cancer cells and shrink the size of the tumor. This relies on the use of x-rays and other high-energy rays generated by a machine outside of the body.
• Chemotherapy to kill cancer cells and shrink tumors. This method relies on powerful anti-cancer drugs that may be taken by mouth or injected into a vein or muscle. Chemotherapy given after the cancer has been surgically removed is called adjuvant chemotherapy. Chemotherapy given before surgery to shrink the tumor is called neoadjuvent chemotherapy.
• Bone marrow transplantation: In this procedure, diseased marrow is killed with radiation and/or chemotherapy, and replaced with healthy marrow.

Back to top

Treatment by Stage

Localized resectable neuroblastoma

• 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 the child is younger than 1 year of age, treatment may include one of the following:

• Surgery to remove the cancer.
• Chemotherapy

If the 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.

Back to top

Disseminated neuroblastoma (Stage IV)

• Multi-drug chemotherapy with surgery and radiation therapy
• Chemotherapy followed by peripheral stem cell transplantation and 13-cis retinoic acid or possibly immunotherapy
• A clinical trial of new methods of treatment

Stage IVS neuroblastoma

Children with this special type of neuroblastoma may not require therapy.

Recurrent neuroblastoma

Choosing a treatment for this condition depends on several things, including the location and extent of the recurrence, or progression; previous therapy received; and patient's overall health and response to previous treatments. A clinical trial may be appropriate.

Back to top

Clinical Trials

The Masonic Cancer Center is a source of innovative new therapies for pediatric cancers, often referred to as Phase 1 or 2 clinical trials. We have compiled detailed information, including eligibility criteria, for some of the advanced therapies available through the Cancer Center.

• Detailed information on selected clinical trials available for Neuroblastoma
• View all childhood cancer clinical trials available through the Masonic Cancer Center.

If you have questions about clinical trials, contact the Masonic Cancer Center's Cancer Information Line.

Back to top