Understanding Neuroblastoma Risk Assessments & Treatment Recommendations

Determining a child’s risk of neuroblastoma recurrence is an important part of their diagnosis and treatment. Assessment of risk ultimately dictates the treatment protocol your doctor will recommend.

Neuroblastoma Low Risk Assessment

Children at low risk often require surgery as their only treatment. Even in cases where some neuroblastoma is left behind after surgery, the child can usually be watched carefully without further treatment because the remaining tumor will often mature or go away on its own.

Chemotherapy is typically given after surgery if less than half the tumor can be removed. A common chemotherapy regimen is a combination of carboplatin, cyclophosphamide, doxorubicin, and etoposide. But other combinations may be used.

For those few children that have symptoms from a tumor that can't safely be treated right away with surgery, a short course of chemotherapy might be given. For example, if the tumor is pressing on the spinal cord or affecting breathing, chemotherapy may be used to shrink the tumor to control the symptoms. Radiation therapy may be needed if chemotherapy doesn't shrink the tumor fast enough.

Infants with 4S disease and no symptoms can often be watched carefully with no treatment, because these cancers often mature or go away on their own.

Neuroblastoma Intermediate Risk Assessment

Surgery is an important part of treatment for children at intermediate risk, but it is rarely enough on its own. Children are typically given 4 to 8 cycles (about 12 to 24 weeks) of chemotherapy before or after surgery. The chemotherapy drugs used are usually the same as for low-risk disease. Radiation therapy may be used if chemotherapy is not effective.

If chemotherapy is used after surgery, a "second look surgery" may be done to see if there is any cancer remaining and, if there is, remove it if possible. This may be followed by radiation therapy, if needed.

Neuroblastoma High Risk Assessment

Children at high risk require more aggressive treatment, which often includes a combination of chemotherapy, surgery, and radiation. In many cases, high-dose chemotherapy followed by a stem cell transplant is used. Surgery and/or radiation may be part of this treatment regimen. The retinoid drug 13-cis-retinoic acid (isotretinoin) is often given for 6 months after other treatments are completed. Immunotherapy with a monoclonal antibody (ch14.18) and cytokines (immune system-activating hormones) is often given as well.