Children with neuroblastoma have a wide range of presenting signs and symptoms. This cancer must be considered in every child with hepatomegaly, skin nodules, periorbital ecchymosis, bone pain, Horner's syndrome, opsoclonus-myoclonus, and/or transverse myelitis. Neuroblastoma is characterized by a diversity of clinical behavior, but to a large extent, outcome can be predicted by the stage of disease, the age at diagnosis, and the presence or absence of MYCN amplification. Additional factors, such as tumor pathology, DNA content, genetic abnormalities, and the presence of clinically threatening symptoms, have also been found to have prognostic value. Most countries have developed treatment strategies that are tailored according to patient risk; however, currently uniform criteria for risk-group stratification do not exist. An International Neuroblastoma Risk Classification System is needed so that treatment protocols performed in different regions of the world can be compared and optimal therapeutic strategies for patients with low-, intermediate-, and high-risk disease can be identified.

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