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Recurrent Neuroblastoma

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    Recurrent Neuroblastoma in Patients Initially Classified as Low Risk

    (Risk categories are defined in Table 1 in the Stage Information section of this summary.)

    Local/regional recurrence

    Local regional recurrent cancer is resected if possible:

    1. 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.
    2. 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 as used in prior COG trials (COG-P9641 and COG-A3961). Older children with local recurrence with either unfavorable INPC 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.[12] These modalities are commonly employed in the treatment of patients with a recurrence that augurs a poor prognosis.

    Metastatic recurrence

    Metastatic recurrent or progressive neuroblastoma in an infant initially categorized as low risk (see Table 1 in the Stage Information section of the summary) and younger than 1 year at recurrence, whether the patient has INSS stage 1, 2, or 4S at the time of diagnosis, 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 with completely favorable biology occurs more than 3 months after diagnosis or not in a 4S pattern, then the primary tumor is resected if possible and 12 to 24 weeks of chemotherapy are given, depending on response.
    3. If the tumor in the infant with metastatic recurrence or progression has unfavorable INPC classification and/or is diploid, the primary tumor is resected if possible and 24 weeks of chemotherapy is given.
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