What is Neuroblastoma?

Neuroblastoma is a children’s cancer that occurs in infants and young children and is rarely found in children older than 10 years of age. In the United States, approximately 700 children and adolescents younger than 20 years of age are diagnosed with tumors of the sympathetic nervous system each year, of which approximately 650 are neuroblastomas. More alarming, though, is that over 97% of sympathetic nervous system tumors are neuroblastomas.

The cells of this cancer usually resemble very primitive developing nerve cells found in an embryo or fetus. (The term neuro indicates "nerves," while blastoma refers to a cancer that affects immature or developing cells). These solid tumors, which take the form of a lump or mass, commonly begin in one of the adrenal glands, though they can also develop in nerve tissues in the neck, chest, abdomen, or pelvis.

Prognosis (chance of recovery) and treatment options depend on the age of the child when diagnosed, stage of disease, location of the tumor in the body, and characteristics of the tumor. The average age at diagnosis is two. It is extremely difficult to diagnose in small children, and once diagnosed, its progression is often rapid, and very painful. Neuroblastoma is responsible for over 15% of all cancer deaths.

Why Early Detection and Diagnosis are Important

Neuroblastoma is often first discovered when parents or a physician feel a mass or tumor somewhere in the child's body. Sometimes symptoms resemble those of many more common childhood illnesses, such as bruising, mild flu symptoms, diarrhea, and loss of appetite. Sometimes there are no outward symptoms. More acute symptoms can include bone pain, hypertension, anemia, skin nodules, a pronounced limp, or a refusal to walk. Because symptoms are so unclear, half of all neuroblastomas have already spread to other parts of the body by the time suspicions are raised and a diagnosis is made.

For all stages, infants less than one year old have a much better chance for remission and survival than older children. Early diagnosis is one of our best weapons in the war against Neuroblastoma. Children under the age of one have a cure rate as high as 90%. For children over the age of one, statistics are much worse. Between 1985-1994, for children aged 1 to 4 years at diagnosis, the 5-year survival rate was 55%; for children 5 years and older, 40%. More alarmingly, though, are results from a St. Jude study that reviewed medical records of patients older than 1 year who had advanced (stage IV) Neuroblastoma, the most common extracranial (not involving the brain) solid tumor of childhood. The investigators found that the 5-year survival estimates were at about 30%. A report on this study appeared in the December 15, 2005, edition of the journal CANCER.

Why More Research is Needed

• Neuroblastoma is a complex tumor of the sympathetic nervous system, a condition that has been known for 35 years. Still, there is very little known about why Neuroblastoma occurs, or what factors increase risk for occurrence.
• Neuroblastoma is one of the childhood cancers most resistant to treatment.
• While chemotherapy has been generally effective for controlling disease with high-risk Neuroblastoma, more tumor-selective and more effective forms of therapy are urgently needed for cure.
• Nearly 70% of those children first diagnosed have disease that has already metastasized or spread to other parts of the body.

Neuroblastoma and Childhood Cancer

Did you know that...

Cancer is the # 1 cause of death by disease in children. (CureSearch)

In the United States in 2007, approximately 10,400 children under age 15 were diagnosed with cancer and about 1,545 children will die from the disease. (National Cancer Institute, U.S. National Institutes of Health)

Neuroblastoma is by far the most common cancer in infants and the fourth most common type of cancer in children. There are approximately 650 new cases of Neuroblastoma each year in the United States. This number has held steady for many years. In as many as 6 or 7 of 10 cases, the disease is not diagnosed until it has already spread (metastasized). (American Cancer Society)

The cause of Neuroblastoma is unknown. (Memorial Sloan-Kettering Cancer Center)

Neuroblastoma is one of the childhood cancers most resistant to treatment. (The Children’s Hospital of Philadelphia)

Approximately 50% of children with Neuroblastoma will have high-risk disease, which often reoccurs, or relapses, or becomes resistant to standard therapies and gets worse. (NANT (New Approaches to Neuroblastoma Therapy))

Neuroblastomas are highly diverse in their behavior. (Memorial Sloan-Kettering Cancer Center)

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

• Age of the child when diagnosed
• Stage of the cancer
• Location of the tumor in the body
• Tumor histology (the shape, function, and structure of the tumor cells)

By the time Neuroblastoma is diagnosed, the disease has usually spread to other parts of the body, most often to the lymph nodes, bones, bone marrow, liver and skin. (National Cancer Institute)

Staging is the most important factor in estimating the prognosis and choosing the most appropriate treatment. (American Cancer Society)

Neuroblastoma Information Resources

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