In general the treatments used will follow the risk groups.

Low risk: Children at low risk usually require only surgery. Infants with some residual neuroblastoma after surgery can often be watched without treatment because the tumor will often spontaneously disappear. A short course of chemotherapy might be given to those few children that have symptoms from the tumor. For example, if the tumor is pressing on the spinal cord or interfering with breathing, chemotherapy may be required to control the symptoms. Chemotherapy is also given if less than half the tumor can be surgically removed. Infants with 4S disease and no symptoms may be watched with no treatment, because often the cancer disappears spontaneously. One typical chemotherapy regimen is a combination of carboplatin, cyclophosphamide, etoposide and doxorubicin. But other combinations may be used.

Intermediate risk: For children at intermediate risk, 4 to 8 cycles (about 12-24 weeks) of chemotherapy are usually given before or after surgery to control the disease. The chemotherapy is the same as for low risk disease. A second surgery called “second look surgery” or radiation therapy may also be required. The role of second look surgery would be to see if there is any cancer remaining and, if there is, remove it if possible. This may then be followed by the radiation therapy.

High risk: For children at high risk, very intensive chemotherapy along with blood-forming stem cell transplant (bone marrow or peripheral blood) is being used. Surgery and/or radiation may be part of this treatment regimen. Biologic agents such as 13-cis retinoic acid are often given for 6 months after therapy is completed.

Unfortunately, some children will have a recurrence of the disease after initial therapy. Treatment at this point will depend on many factors, including the initial risk group and where the cancer recurs. Chemotherapy, surgery, radiation therapy or even simple observation without treatment may be appropriate, depending on the circumstances. In those cases where the neuroblastoma cells uptake the MIBG, treatment with high doses of this radioactive molecule may be given. The dose is so high that the patient usually requires blood-forming stem cell transplant after that. Another option would be retinoic acid.

Revised: 08/04/2006