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Neuroblastoma

- Summary
- About neuroblastoma
- Potential causes
- Signs and symptoms
- Diagnosis methods
- Treatment options
- Prevention methods
- Staging
- Questions for your doctor

Reviewed By:
Martin E. Liebling, M.D., FACP
Mark Oren, M.D., FACP

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Prevention methods for neuroblastoma

There is no known way to prevent neuroblastoma. Because genetic factors can be associated with the disease, people with a family history of cancers, especially childhood cancers, should discuss genetic testing with their children’s physician.

Researchers have conducted tests to determine if large amounts of catecholamines in the urine of infants could help in early detection of neuroblastoma. However, the test results revealed too large a number of positive growths. The large number may be due to neuroblast cells that release high levels of catecholamines, which regress and die independently. For this reason, urine tests have not been useful for early detection, prevention or treatment of neuroblastoma.

Staging neuroblastoma

After the diagnosis of neuroblastoma, tests are performed to determine the stage or spread of the cancer. Staging is the process used to determine the extent of growth of the primary tumor and the metastasis (spread) to other tissues in the body.

Staging is an important tool for determining the best treatment path and prognosis for recovery. Neuroblastoma is staged in accordance with the International Neuroblastoma Staging System guidelines:

Stage 1. The tumor is localized on one side of the body. All of the visible tumor can be completely removed with surgery. The lymph nodes within the tumor may have cancer cells but those outside of the tumor are not cancerous.
Stage 2A. The tumor is localized on one side of the body, but cannot be completely removed with surgery. The lymph nodes within the tumor may have cancer cells but those outside of the tumor are not cancerous.
Stage 2B. The tumor is localized on one side of the body, and may or may not be completely removed with surgery. The cancer has spread to nearby lymph nodes only on the same side of the body as the original tumor.
Stage 3. The tumor has spread across the midline of the body and cannot be completely removed by surgery and may have spread to nearby lymph nodes. Or, the tumor is on one side of the body and has spread to lymph nodes on the other side of the body. Or, the tumor is in the middle of the body and has spread to the lymph nodes on both sides of the body and cannot be surgically removed.
Stage 4. The primary tumor has spread to distant lymph nodes, bone, bone marrow, liver, skin or other organs and the patient does not meet the criteria for stage 4S.
Stage 4S (also called “special” neuroblastoma). Occurs in infants less than one year old. The tumor is localized on one side of the body and may have invaded lymph nodes on the affected side of the body. The cancer has spread to the skin, liver or bone marrow with no more than 10 percent cancerous marrow cells and no bone damage. Children in this category tend to have better survival rates than older children with metastatic disease. Overall, survival is about 85 percent.
Recurrent. The cancer has returned after being treated. It may return to the original location or another part of the body.

After diagnosis, patients are placed in a risk group that helps determine their treatment options and prognosis. Factors included in determining the risk are the age of the child, types of cells involved, their rate and range of growth and DNA factors. According to the American Cancer Society (ACS), prognostic groups and a description of treatment for these groups include:

Low risk. Surgery alone is usually required. Chemotherapy may be used to control symptoms.
Intermediate risk. Chemotherapy is administered prior to surgery. Radiation may also be needed.
High risk. Very intensive chemotherapy, including stem cell transplant (bone marrow or peripheral blood), is required. Surgery and/or radiation may be used. Biological agents are given after therapy is complete.

The following are 5-year survival rates for neuroblastoma, according to the ACS: