Neurobastoma is a small round blue cell tumor that uniquely has the ability to mature and regress, either spontaneously or in response to treatment, or conversely, become undifferentiated and highly malignant. Due to this, treatment ranges from simple observation in select infants to aggressive multimodal therapy including chemotherapy, surgical resection, myeloablative therapy with stem cell rescue, radiation therapy, and immunotherapy. Tumor staging and risk classification for neuroblastoma continue to evolve as new genomic prognostic markers are identified and efforts continue to unify risk-based treatment strategies worldwide. Surgical resection plays an important role in the local control of neuroblastoma. In patient with high-risk neuroblastoma, neoadjuvant chemotherapy followed by primary tumor resection of ≥90% has been shown to improve local control and may improve both event free and overall survival. In low- and intermediate-risk patients, survival is high and therefore, efforts are focused on therapy reductions in order to diminish treatment related complications. Unfortunately, the majority of neuroblastoma patients present with high-risk disease in which outcomes have improved but remain poor.

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Neuroblastoma

  • Dave R. Lal,
  • Andrew M. Davidoff

摘要

Neurobastoma is a small round blue cell tumor that uniquely has the ability to mature and regress, either spontaneously or in response to treatment, or conversely, become undifferentiated and highly malignant. Due to this, treatment ranges from simple observation in select infants to aggressive multimodal therapy including chemotherapy, surgical resection, myeloablative therapy with stem cell rescue, radiation therapy, and immunotherapy. Tumor staging and risk classification for neuroblastoma continue to evolve as new genomic prognostic markers are identified and efforts continue to unify risk-based treatment strategies worldwide. Surgical resection plays an important role in the local control of neuroblastoma. In patient with high-risk neuroblastoma, neoadjuvant chemotherapy followed by primary tumor resection of ≥90% has been shown to improve local control and may improve both event free and overall survival. In low- and intermediate-risk patients, survival is high and therefore, efforts are focused on therapy reductions in order to diminish treatment related complications. Unfortunately, the majority of neuroblastoma patients present with high-risk disease in which outcomes have improved but remain poor.