Introduction

Intermediate-risk neuroblastoma is a clinically and biologically heterogeneous entity. According to the current Children's Oncology Group (COG) Neuroblastoma Risk Stratification System (see Chap. 7), this grouping includes infants with INSS stages 3 or 4 tumors that lack MYCN amplification, infants with stage-4S disease with normal MYCN copy number and either unfavorable histology or diploidy, and children >1 year of age with favorable histology stage-3 tumors that lack MYCN amplification (Table 11.2.1). Based on these clinical and biologic criteria, approximately 15% of all patients diagnosed with neuroblastoma are classified as intermediate risk (Table 11.2.2). Previous clinical trials have shown that >85 % of these patients can be cured with moderate-dose chemotherapy and surgery (Bowman et al. 1997; Garaventa et al. 2002; Matthay et al. 1998; Rubie et al. 2001; Schmidt et al. 2000; Strother et al. 1997) (Table 11.2.2 and Figure 11.2.1); thus, these patients stand apart from those with high-risk disease, who have long-term survival rates of <30 % even with intensive multi-modality therapy (see the present chapter), and from children with low-risk neuroblas-toma, who are usually cured with surgery alone (see the present chapter).

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