Neuroblastoma is a malignant tumor of neural crest origin that may arise anywhere along the sympathetic ganglia or within the adrenal medulla. The median age of diagnosis is 2 years; however, occurrence is skewed toward younger children, with nearly 35% of cases occurring under 1 year of age and the remainder under 10 years of age. Seventy-five percent of neuroblastomas originate within the abdomen or pelvis, and half of these occur within the adrenal medulla, whereas 20% originate within the posterior mediastinum and 5% within the neck.

The overall incidence of neuroblastoma in the unscreened population is 1 case per 10,000 persons, with approximately 525 newly diagnosed cases in the United States each year. In Japan and Quebec Province, where the population is screened at 3 weeks and 6 months of age using a urinary assay for urinary catecholamine homovanillic acid (HVA) and vanillylmandelic acid (VMA) produced by 85% to 90% of neuroblastomas, the incidence is closer to 1 case per 7000 individuals.<sup>41 Sawada T., Nakata T., Takasugi N. , e al. Mass screening for neuroblastoma in infants in Japan Lancet 1984 ;  2 : 271-273  [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 50 Woods W.G., Tuchman M., Robison L.L. A population-based study of the usefulness of screening for neuroblastoma Lancet 1996 ;  348 : 1682-1687  [cross-ref]

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Neuroblastoma is an enigmatic tumor capable of rapid progression in some children and spontaneous resolution in other children usually less than 1 year of age.<sup>3 Beckwith J.B., Perrin E.V. In situ neuroblastomas: A contribution to the natural history of neural crest tumors Am J Pathol 1963 ;  43 : 1089

Cliccare qui per andare alla sezione Riferimenti</sup> Twenty-five percent of cases present with a solitary mass that may be cured by surgical therapy, whereas nearly 60% of cases present with disseminated disease involving most commonly lymph nodes, liver, bone, and bone marrow. In this latter group of children, survival is poor, exemplified by the fact that although neuroblastoma accounts for only 10% of all childhood concerns, it is responsible for 15% of all cancer-related deaths in the pediatric age group.<sup>31 Miller R.W., Young J.L., Novakovic B. Childhood cancer Cancer 1995 ;  75 (suppl) : 395-405  [cross-ref]

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This wide range of clinical behavior reflects the considerable biologic diversity of this tumor. Although progress in the treatment of neuroblastoma has been painstakingly slow, recent advances in molecular genetics and staging have increased understanding of the biologic determinants of the disease and the resultant clinical subsets.<sup>7 Brodeur G.M., Azar C., Brother M. , e al. Neuroblastoma: Effect of genetic factors on prognosis and treatment Cancer 1992 ;  70 : 1685-1694  [cross-ref]

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