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Neuroblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Recurrent Neuroblastoma

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Recurrent Neuroblastoma in Patients Initially Classified as Low Risk

Locoregional recurrence

Standard treatment options for locoregional recurrent neuroblastoma initially classified as low risk include the following:

  1. Surgery followed by observation or chemotherapy.

Surgery followed by observation or chemotherapy

Local or 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 chemotherapy.

Infants younger than 1 year at the time of locoregional 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 chemotherapy.

Chemotherapy may consist 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 as used in prior COG trials (COG-P9641 and COG-A3961). Older children with local recurrence with either unfavorable International Neuroblastoma Pathology Classification at diagnosis or MYCN gene amplification have a poor prognosis and are treated with aggressive combination chemotherapy, offered palliative care, or offered entry onto a clinical trial.

Evidence (surgery alone or chemotherapy with or without surgery):

  1. A COG study of treatment of low-risk patients with stage 1, 2A, 2B, and 4S neuroblastoma enrolled 915 patients, 800 of whom were asymptomatic and were treated with surgery alone followed by observation. The others received chemotherapy with or without surgery.
    • About 10% of patients developed progressive or recurrent tumor. The majority of the recurrences were treated on study with surgery alone or moderate chemotherapy with or without surgery, and most were salvaged as demonstrated by the EFS (89%) and OS (97%) rates at 5 years.[9]

Metastatic recurrence

Standard treatment options for metastatic recurrent neuroblastoma initially classified as low risk include the following:

  1. Observation.
  2. Surgery followed by chemotherapy.

Metastatic recurrent or progressive neuroblastoma in an infant initially categorized as low risk and younger than 1 year at recurrence may be treated according to tumor biology as defined in the prior COG trials (COG-P9641 and COG-A3961):

  1. If the biology is completely favorable, metastasis is in a 4S pattern, and the recurrence or progression is within 3 months of diagnosis, the patient is observed systematically.
  2. If the metastatic progression or recurrence occurs more than 3 months after diagnosis or not in a 4S pattern, then the primary tumor is resected if possible and chemotherapy is given.

    Chemotherapy may consist 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, as used in prior COG trials (COG-P9641 and COG-A3961).

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