25 - Neuroblastoma

Summary

Neuroblastoma (NB) originates from neural crest precursors in the adrenal medulla, the sympathetic ganglia in the neck, mediastinum, retroperitoneum, or pelvis. It is the most common extracranial pediatric solid tumor and the most common neoplasm among infants. More than 90 percent of the more than 700 cases diagnosed yearly in the United States are in children younger than five years of age.

NB has a notorious reputation among solid tumors of childhood because of its massive and widespread tumor burden. Stage for stage, however, this embryonal neoplasm of the sympathetic nervous system has become one of the most curable pediatric solid tumors. In fact, more than 90 percent of patients with localized NBs, including those with tumors spreading to regional lymph nodes, will survive, often with little or no cytotoxic therapy. Cure rates of metastatic NB exceed 90 percent in infants (usually treated with low-dose chemotherapy) and approximately 25 percent in toddlers. In contrast to NB, osteomedullary metastases result in less than a 5 percent chance of cure for other pediatric solid tumors. The long-held view that many cases spontaneously regress or mature into asymptomatic ganglioneuromas was confirmed by the findings of urine catecholamine screening programs in infants. These programs yielded 50 percent to 100 percent more cases than the unscreened population. Most patients identified had the low-risk forms of the disease, and the incidence of high-risk disease was not reduced.

Environmental causative factors have not been identified, nor has NB been significantly associated with any other disease or condition.