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Neuroblastoma

What is neuroblastoma?

Neuroblastoma is a cancerous tumor that begins in nerve tissue of infants and very young children. The abnormal cells are often found in the nerve tissue that is present in the unborn baby and later develops into a detectable tumor. Neuroblastoma is rare in children older than 10 years of age, however, it does occur occasionally in adults.

The tumor usually begins in the tissues of the adrenal gland found in the abdomen, but may also begin in nerve tissue in the neck, chest, and/or pelvis. The adrenal glands are positioned on top of the kidneys. These glands secrete hormones and other important substances that are required for normal functions in the body such as the nervous system.

In the US, approximately 650 children are diagnosed with neuroblastoma each year. It is often present at birth, but not detected until the tumor begins to grow and compress the surrounding organs. Most children affected by neuroblastoma have been diagnosed before the age of 5. In rare cases, neuroblastoma can be detected before birth by a fetal ultrasound.  It is the most common solid tumor cancer in childhood.

Neuroblastoma cancer cells can spread (metastasize) quickly to other areas of the body (i.e., lymph nodes, liver, lungs, bones, central nervous system, and bone marrow). Approximately 70 percent of all children diagnosed with neuroblastoma will have some metastatic disease.

What causes neuroblastoma?

The only risk factor that has been established for neuroblastoma is heredity. Recent research indicates that a common genetic variation of the gene 6p22 on chromosome 6 doubles the risk of this disease. Also, having this particular variation increases the chance that a child will develop a more aggressive form of the disease.

The average age at diagnosis in genetically linked cases is around nine months. When the cancer presents in several different areas of the body at once, this suggests it may be a genetically inherited cancer.

What are the symptoms of neuroblastoma?

The following are the most common symptoms of neuroblastoma. However, each child may experience symptoms differently. The symptoms of neuroblastoma vary greatly depending on size, location, and spread of the tumor. Symptoms may include:

The symptoms of neuroblastoma may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.

How is neuroblastoma diagnosed?

In addition to a complete medical and physical examination, diagnostic procedures for neuroblastoma may include the following:

Diagnosing neuroblastoma also involves staging and classifying the disease which determines treatment options and prognosis. Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging systems that can be used for neuroblastoma. Always consult your child's physician for information on staging. One method of staging neuroblastoma is the following:

Treatment for neuroblastoma:

Specific treatment for neuroblastoma will be determined by your child's physician based on:

Treatment may include (alone or in combination):

Treatment options should be discussed with your child's physician.

Long-term outlook for a child with neuroblastoma:

Prognosis greatly depends on:

As with any cancer, prognosis and long-term survival can vary greatly from individual to individual. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with neuroblastoma. Side effects of radiation and chemotherapy, as well as recurrence of the disease, can occur in survivors of neuroblastoma. New methods are continually being discovered to improve treatment and to decrease side effects.

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