Brain Cancer Health Center
Neuroblastoma Treatment - Recurrent Neuroblastoma
The prognosis and treatment of recurrent or progressive neuroblastoma depends on many factors including initial stage, tumor biological characteristics at recurrence, the site and extent of the recurrence or progression, previous treatment, and individual patient considerations. In selected patients originally diagnosed with low- or intermediate-risk disease, recurrence may be treated successfully with limited intervention. When neuroblastoma recurs in a child originally diagnosed with high-risk disease and is widespread, the prognosis is usually poor despite additional intensive therapy.[1,2,3] The combination of cyclophosphamide plus topotecan has been active in patients with recurrent or refractory disease who have not received topotecan previously.[4] 131-I-metaiodobenzylguanidine (131-I-MIBG) therapy is also active in patients with recurrent or refractory neuroblastoma.[5] Clinical trials are appropriate and should be considered. Information about ongoing clinical trials is available from the NCI Web site.
Central nervous system (CNS) involvement, though rare at initial presentation, may occur in 5% to 10% of patients with recurrent neuroblastoma. Inward compression of the brain from cranial metastases can occur, and rarely meningeal and isolated intracranial metastases occur. Early recognition and treatment of CNS involvement may result in reduced neurologic impairment.[6,7]
Children's Oncology Group Treatment Plan
In North America, the Children's Oncology Group (COG) is investigating a risk-based neuroblastoma treatment plan that assigns all patients to a low-, intermediate-, or high-risk group based on age, International Neuroblastoma Staging System (INSS) stage, and tumor biology (i.e., MYCN gene amplification, Shimada classification, and DNA ploidy).[8] Treatment of recurrent disease is determined by the risk group at the time of diagnosis (refer to the table in the Stage Information section of this summary), extent of disease at recurrence, patient age at recurrence, and the tumor biology at recurrence. If tumor is unavailable for biological studies at recurrence, the biology of the tumor at the time of diagnosis is used to help determine treatment.
Recurrent Neuroblastoma in Patients Initially Classified as Low Risk
(Risk categories are defined in the table in the Stage Information section of this summary.)
Local/regional recurrence
Local regional recurrent cancer is resected if possible:
- Those with favorable biology and regional recurrence more than 3 months after completion of planned treatment are observed if resection of the recurrence is total or near total (=90% resection). Those with favorable biology and a less than near-total resection are treated with 12 weeks of chemotherapy.
- Infants younger than 1 year at the time of local/regional recurrence whose tumors have any unfavorable biologic properties are observed if resection is total or near total. If the resection is less than near total, these same infants are treated with 24 weeks of chemotherapy.
Chemotherapy consists of moderate doses of carboplatin, cyclophosphamide, doxorubicin, and etoposide. The cumulative dose of each agent is kept low to minimize permanent injury from the chemotherapy regimen (COG-P9641). Older children with local recurrence with either unfavorable Shimada classification or MYCN gene amplification have a poor prognosis and should be treated with an aggressive regimen of combination chemotherapy consisting of very high doses of the drugs listed above, and often also including ifosfamide and high-dose cisplatin. Both myeloablative therapy and postchemotherapy retinoic acid may improve outcome of newly diagnosed high-risk patients with a poor prognosis.[9] These modalities are commonly employed in the treatment of patients with a recurrence that augurs a poor prognosis.
WebMD Public Information from the National Cancer Institute
This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER
Neuroblastoma Treatment Topics
Important Safety Information
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