Treatment for neuroblastoma is largely based on which risk group a child falls into.

Low risk

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.

Intermediate risk

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.

High risk

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.

Recurrent neuroblastoma

Unfortunately, sometimes neuroblastoma can come back (known as a recurrence or relapse) after initial therapy. Treatment at this point will depend on many factors, including the initial risk group and where the cancer recurs.

For low- and intermediate-risk neuroblastomas that recur in the same area where they started, surgery with or without chemotherapy may be appropriate.

For higher-risk cancers or those that recur in distant parts of the body, treatment is usually more intense, and may include a combination of chemotherapy, surgery, and radiation therapy (such as MIBG radiotherapy). In some relapsed cases, intensive treatment with high-dose chemotherapy/radiation therapy, followed by a donor stem cell transplant, may be used. Because these cancers can be hard to treat, clinical trials of experimental treatments, such as monoclonal antibodies or new anti-cancer drugs, may be another reasonable option. (See "What's new in neuroblastoma research and treatment?")

Last Medical Review: 11/23/2009
Last Revised: 11/23/2009