Neuroblastoma is the fourth most common pediatric malignancy, comprising about 8% of all cancer diagnosed in children under 15 years of age.Yet it is responsible for a disproportionate number of deaths in children from cancer, approximately 15% of all pediatric cancer deaths. Neuroblastoma is a very heterogeneous disease: tumors can spontaneously regress or mature or display a very aggressive, malignant phenotype. And so, it has been of great interest to both clinicians and basic scientists. Progress in molecular and cellular biology has contributed greatly to a better understanding of neuroblastoma. In fact, neuroblastoma has served as a model for a molecular approach to treating patients with cancer, as increasing evidence indicates that the biologic and molecular features of neuroblastoma are highly predictive of clinical behavior. Therefore, current treatment of children with neuroblastoma is based not only on stage but also on risk stratification. This risk stratification takes into account both clinical and biologic variables predictive of disease relapse, with the status of the MYCN proto-oncogene (amplified or non-amplified) within the tumor cells currently being the most powerful biologic factor.Overall, approximately 25% of primary neuroblastomas in children have MYCN amplification, with MYCN amplification being present in 40% with advanced disease.
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