Treatment of neuroblastoma is aimed at increasing a child's survival, reducing the risk of recurrence and ensuring an as near-normal growth as possible of the child with the condition. Treatment involves a multidisciplinary team that may include a pediatric oncologist, a psychologist, social workers, rehabilitation specialists and oncology nurses, for example.
Treatment with surgery is almost always successful if the tumour is detected when an infant is under 18 months of age or when the cancer is localized to its site of origin. Survival, however, is poor in children who are aged over 18 months or who have more advanced cancer that has spread to other organs.
The features of the cancer are usually determined at the outset so a child can be assigned to a risk group and the appropriate therapy planned. The factors taken into consideration when deciding on the child's risk category include:
Age of the child
Extent the cancer has spread
Appearance of the cancer cells under the microscope
Genetic features such as number of chromosomes and N-myc oncogene amplification
Low risk patients can be successfully treated using surgery alone, while intermediate risk patients require both surgery and chemotherapy for treatment. High risk neuroblastomas need to be treated using surgery, radiation and chemotherapy. Stem cell transplant and biological-based therapies such as retinoids or immune modulating agents may also be used.
Surgery for neuroblastoma
Surgical techniques may be employed to confirm a diagnosis and/or to remove a tumor. For smaller tumors that have not yet begun to spread, surgery may be the only therapy required.
Chemotherapy for neuroblastoma
A variety of different chemotherapy agents may be used in combination to treat neuroblastoma. Examples of drugs that may be used include carboplatin, vincistrine, cyclophosphamide, etoposide, doxorubicin and melphalan.
Chemotherapy may be given either before surgery (neoadjuvant chemotherapy) or after surgery (adjuvant chemotherapy). The most common therapies used are doxorubicin, carboplatin, etoposide and cyclophosphamide.
Retinoid therapy for neuroblastoma
Retinoids are derivatives of Vitamin A and have been found to be effective in treating neuroblastomas. The use of 13-cis-retinoic acid or isotretinoin in high risk neuroblastoma has reduced the risk of recurrence after treatment with high-dose chemotherapy and stem cell transplant. Treatment is usually taken in cycles of twice daily for a fortnight followed by two weeks off, over a six month period.
Radiation therapy for neuroblastoma
Radiation therapy involves the use of high-energy beams to destroy cancer cells. It is used in combination with surgery and/or chemotherapy.
Immunotherapy for neuroblastoma
Monoclonal antibodies, which are designed to target cancer cells, can be injected into the body. These immune system proteins seek out and attach to cancer cells which they then destroy.
A monoclonal antibody called ch14.18, for example, attaches to the ganglioside GD2 that is often present on neuroblastoma cells. It can be given along with cytokines (chemical cell messengers) that provide signals to the immune system to help identify and destroy the neuroblastoma cells.
Reviewed by Sally Robertson, BSc