ABSTRACT

The annual incidence of neuroblastoma is about one case per 100 000 children, thereby, accounting for about 8% of all malignancies in those younger than 15 years of age. Complete resection of neuroblastoma offers definitive therapy with a generally excellent outcome for most patients with localized disease, although benefit of complete tumor removal may be overestimated because of possibility that localized neuroblastomas may undergo spontaneous maturation or regression. The role of surgery is even less clear in curative treatment of patients with high-risk neuroblastoma and attempts at resection can be associated with significant complications. The diagnosis of neuroblastoma is generally made by histopathologic evaluation of primary or metastatic tumor tissue, or by demonstration of tumor cells in bone marrow together with elevated levels of catecholamine metabolites in urine. The general approach to treating patients with high-risk neuroblastoma includes intensive induction chemotherapy, myeloablative consolidation therapy with stem cell rescue, and targeted therapy, including differentiating agents and immunotherapy, for minimal residual disease.