Neuroblastoma is the most common extracranial solid tumor in children and can arise from the sympathetic chain anywhere with variable presentation. Disease stratification using factors like age, stage, histopathology, avian myelocytomatosis viral oncogene neuroblastoma derived homolog (MYCN) amplification, and 11q aberrations is important during presentation. Low-risk disease which includes stage L1 and asymptomatic stage L2 has excellent outcomes with surgery alone. The intermediate-risk disease requires neoadjuvant chemotherapy prior to surgery. This entity includes large unresectable stage L2 and stage M neuroblastoma. Asymptomatic stage MS disease and perinatal neuroblastoma arising from the adrenals can be managed with close observation alone. However, organ dysfunction and children below 3 months might require chemotherapy cycles in MS disease. Paraspinal neuroblastomas are to be treated as an emergency. High-risk neuroblastoma has a worse outcome which includes MYCN amplified tumors and older children with stage M disease. However, the outcomes have improved with a multimodality approach using immunotherapy and autologous stem cell transplant. This chapter gives an overview of the approach toward patients of neuroblastoma including perioperative considerations.

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Neuroblastoma

  • Nitin J. Peters,
  • Saswati Behera,
  • Subhasis Roy Choudhury

摘要

Neuroblastoma is the most common extracranial solid tumor in children and can arise from the sympathetic chain anywhere with variable presentation. Disease stratification using factors like age, stage, histopathology, avian myelocytomatosis viral oncogene neuroblastoma derived homolog (MYCN) amplification, and 11q aberrations is important during presentation. Low-risk disease which includes stage L1 and asymptomatic stage L2 has excellent outcomes with surgery alone. The intermediate-risk disease requires neoadjuvant chemotherapy prior to surgery. This entity includes large unresectable stage L2 and stage M neuroblastoma. Asymptomatic stage MS disease and perinatal neuroblastoma arising from the adrenals can be managed with close observation alone. However, organ dysfunction and children below 3 months might require chemotherapy cycles in MS disease. Paraspinal neuroblastomas are to be treated as an emergency. High-risk neuroblastoma has a worse outcome which includes MYCN amplified tumors and older children with stage M disease. However, the outcomes have improved with a multimodality approach using immunotherapy and autologous stem cell transplant. This chapter gives an overview of the approach toward patients of neuroblastoma including perioperative considerations.