ABSTRACT

Cancer in the newborn is relatively rare, comprising only about 2.5" of all childhood malignancies; the majority of tumors in the newborn are benign lesions. Neuroblastoma is a heterogeneous disease; tumors can spontaneously regress or mature without treatment, or display a very aggressive, malignant phenotype. Neuroblastoma is an embryonal tumor that arises from neural crest cells. During normal development, neural crest cells migrate from the developing neural tube to form the sympathetic nervous system. Neuroblastoma generally occurs sporadically, but familial neuroblastoma does occur in about 2" of the cases. Although there is no single genetic abnormality or initiating event common to all neuroblastomas, a number of different genetic alterations have been identified that provide powerful prognostic information and play crucial roles in risk assessment and treatment planning. When symptomatic, children with neuroblastoma usually present with signs and symptoms that reflect the primary site and extent of disease.