Disease Information

Solid Tumor: Neuroblastoma

Alternate Names: None

Definition

• Neuroblastoma is the most common tumor in infants younger than 1 year of age and a common solid tumor of childhood.
• Most children are diagnosed by 2 years of age.
• Tumors originate from neural crest cells (called neuroblasts) in the sympathetic nervous system, which runs from the base of the neck to the tailbone. Accordingly, tumors can appear anywhere along this chain, but are most commonly found in the chest and near the adrenal glands, which are located on top of the kidneys.

Incidence

• Each year, one in 100,000 children in the United States develops neuroblastoma.
• Neuroblastoma accounts for 7-10 percent of childhood cancers.
• Neuroblastoma accounts for 50 percent of all malignancies encountered in infants.

Influencing Factors

• Boys are slightly more likely to develop neuroblastoma than girls.
• A classic genetic basis has not been demonstrated. It is occasionally seen in children with neurofibromatosis, Beckwith-Wiedemann syndrome and a condition of the pancreas known as nesidioblastosis.
• The uniform incidence of neuroblastoma worldwide speaks against environmental etiological factors.
• Infants with neuroblastoma have a much higher chance of cure than older children.
• The excessive presence (amplification) of an oncogene called MYC-N in the tumor cells confers a more aggressive behavior.
• Common symptoms include a hard, painless mass in the neck, an abdominal mass causing pain, irritability and decreased appetite, bone pain from spread of the tumor to the skeleton or weakness or paralysis of the lower legs due to growth into the spinal canal.
• Uncommon presentations include the symptoms of opsomyoclonus and ataxia (loss of coordination and rapid-random eye movements) and Horner’s syndrome (droopy eyelid, a constricted pupil, and inability to produce sweat on one side of the face).

Treatment Strategies

• The patient age, stage (spread of tumor) and certain biologic characteristics are used to categorize patients into one of three groups: low-risk, intermediate-risk or high-risk.
• If the tumor has not spread to other parts of the body, surgery usually is curative.
• If the tumor is causing spinal cord compression, immediate chemotherapy can avoid the use of surgery in the spinal canal.
• Unfortunately, in many cases by the time of diagnosis the disease has usually spread. Chemotherapy is the mainstay of treatment in these cases. The type of chemotherapy and its intensity are determined by the age of the patient and the extent of the disease (risk-factors). Common chemotherapy drugs used to treat neuroblastoma include cyclophosphamide, cisplatin, doxorubicin, etoposide, carboplatin and vincristine. In addition, higher dose chemotherapy and stem cell transplant is commonly used for high-risk patients.
• Radiation therapy in conjunction with chemotherapy is also effective.
• Experimental approaches include chemotherapy with very high doses of new anticancer drugs, bone marrow or stem cell transplantation, treatment with new investigational drugs, tumor vaccines, antibody therapy and other agents that cause the tumor cells to differentiate.

Current Research

• Basic scientists continue to investigate what factors cause neuroblastoma cells to form in the first place.
• A new anti-neuroblastoma antibody is currently under production at St. Jude to provide an additional tool to destroy residual microscopic disease. 
• We are also investigating new ways to remove tumor cells contaminating the bone marrow by using drug combinations or laboratory methods to filter out the tumor cells.
• New treatments with drugs that inhibit cell receptors (on-off switches on tumor cells) or cause cells to undergo differentiation (maturation) are under evaluation in early clinical trials.

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