Low Risk Neuroblastoma Brian H Kushner Susan LCohn 1111 Introduction

Low-risk neuroblastoma is defined as disease that is curable with no or minimal cytotoxic therapy and is strongly associated with spontaneous regression. Approximately 40% of neuroblastoma patients have low-risk disease. According to the Children's Oncology Group (COG) Neuroblastoma Risk-Group Schema criteria, this category includes patients with completely resected localized tumors (stage 1), unresectable unilateral tumors (stage 2A), localized tumors with ipsilateral regional lymph node spread (stage 2B), and infants with unilateral primary tumors with distant disease limited to the bone marrow, liver, and skin (stage 4S; see Chap. 7). Numerous studies have demonstrated excellent survival rates for infants with favorable biology stage-4S neuroblastoma with minimal to no therapy (Guglielmi et al. 1996; Hero et al. 2000; Katzenstein et al. 1998; Nicker-son et al. 2000; Schleiermacher et al. 2003), and most patients with INSS stage-1 and stage-2 disease can be cured with surgery alone (Alvarado et al. 2000; de Bernardi et al. 1995; Evans et al. 1996; Kushner et al. 1996b; Matthay et al. 1989; Nitschke et al. 1988; Perez et al. 2000); however, the resectability of local-regional tumors, which is a defining criterion for INSS stage, is dependent to some extent on subjective factors such as the surgeon's experience and the treating team's commitment to avoiding cytotoxic therapy. Prognostic uncertainty also applies to at least two very rare subtypes currently included in the low-risk category, namely, stage-1 neuroblastoma with MYCN amplification (Cohn et al. 1995) and biologically favorable stage-2 tumors in adolescents (Franks et al. 1997; Gaspar et al. 2003).

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