Changes to this Summary (10/13/2011)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Added Moroz et al. as reference 29.
Added text to state that the central nervous system (CNS) has emerged as a sanctuary site leading to relapse and that current treatment approaches generally include eradicating bulky and microscopic residual disease in the CNS as well as minimal residual systemic disease. Neurosurgical interventions serve to decrease edema, control hemorrhage, and remove bulky tumor prior to starting radiation therapy. Compartmental radioimmunotherapy using intrathecal radioiodinated monoclonal antibodies has been tested in patients with recurrent metastatic CNS neuroblastoma following surgery, craniospinal radiation therapy, and chemotherapy (cited Matthay et al. as reference 8).
Added text about how data from three consecutive German high-risk neuroblastoma trials demonstrated that 23 patients who underwent a second autologous stem cell transplantation (SCT) had a better median survival and 3-year survival rate from recurrence compared with 74 patients who had no second chemotherapy and 135 patients who underwent second-line chemotherapy but did not under SCT. This shows that intensive second-line therapy is feasible, but whether this intense therapy is better than other salvage regimens is unknown (cited Simon et al. as reference 12 and level of evidence 3iiiA). Also added that topotecan alone and in combination with cyclophosphamide or etoposide has been used in patients with recurrent disease who did not receive topotecan initially. High-dose carboplatin-irinotecan-temozolomide has been used in patients resistant or refractory to regimens including topotecan; the combination of irinotecan and temozolomide had a 15% response rate in one study (cited Kushner et al. and Bagatell et al. as references 15 and 16, respectively and levels of evidence 3iiiDiv and 2A, respectively).
Added text to state that for adolescents and young adults with recurrent or refractory neuroblastoma, iodine-131metaiodobenzylguanidine (131I-MIBG) is a highly effective salvage agent and should be considered; intrathecal radioimmunotherapy and whole neuraxis irradiation has been suggested for CNS recurrence (cited Polishchuk et al. and Croog et al. as references 17 and 18, respectively and level of evidence 3iiiA).

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