Intermediate-risk neuroblastoma constitutes a clinically and biologically heterogeneous subset of tumors, which is highly curable with moderate-dose chemotherapy and surgery; thus, current therapeutic strategies are aimed at reducing treatment in an effort to minimize treatment-related toxicities. While on one hand a subset of patients in this group have survived with less or even no therapy, some "high-risk" patients may be safely "down-staged" and managed under the intermediate-risk category (George et al. 2003; Schmidt et al. 2003). Furthermore, achievement of complete remission, i.e., elimination of all evidence of disease, may not be a necessary goal of treatment in intermediate-risk cases. This possibility is based on the limited potential for malignant progression of low- and intermediate-risk neuroblastoma as evidenced by the survival with conservative management of many patients with non-MYCN-am-plified stage 4S and of patients with localized tumors who have gross residual disease following attempted tumor resection (Evans et al. 1996; Matthay et al. 1989; Nickerson et al. 2000). Prospective evaluation of these clinical parameters and further analysis of additional genetic and molecular prognostic variables may enhance our ability to identify children at high vs low risk for disease relapse. Such studies are likely to lead to a further refinement in the current risk-group schema, and hopefully will result in treatment strategies that are optimally tailored for individual children with neuroblastoma.
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