Neuroblastoma comprises a spectrum of tumors that arise from primitive sympathetic ganglion cells and includes neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. This group of tumors is known for a broad spectrum of clinical behaviors ranging from spontaneous regression to widely disseminated, aggressive disease. Tumors can be subdivided into distinct risk categories based on clinical and biological features. Historically, the intensity of treatment has been determined by stage of disease and risk classification such that the majority of patients with low-risk disease have been treated with surgical resection followed by clinical observation. Conversely, patients with high-risk disease require multimodal treatment with chemotherapy, surgery, radiation, and biologic agents.
Neuroblastoma is the third most common childhood cancer after leukemia and brain tumors. From 2003 to 2007, the age-adjusted incidence of neuroblastoma was 10.1 per 1 million children aged 0 to 14 years.1 Of the approximate 650 children diagnosed each year in the United States, the median age is 17.3 months. Neuroblastoma is slightly more common in white versus black infants (1.7 and 1.9 to 1 for male and female infants, respectively), but this difference is not evident in older children.1
The majority of neuroblastomas are sporadic. However, a small subset of children may have concomitant neural crest disorders, including neurofibromatosis, Hirschsprung disease, congenital hypoventilation syndrome, or other predisposing conditions such as Turner syndrome and Beckwith-Wiedemann syndrome. About 1% to 2% of cases are associated with a history of neuroblastoma in immediate or extended ...
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