Neuroblastoma, a malignant embryonal tumor of the neural crest cells, is one of the most common solid extracranial tumors of early childhood (Brodeur et al., 2006). The prevalence of neuroblastoma in children is 7.5 cases /100, 000 infants (Brodeur et al., 2006; Gao et al, 1997; Gurney et al., 1997; Spix et al., 2006). Furthermore, the annual incidence of this tumor is nearly 1.0/100,000 children under the age of 15 years (Ries et al., 2005). Neuroblastoma comprises about 8-10% of all childhood cancers and for approximately 15% of cancer deaths in children (Gao et al., 1997; Ries et al., 2005). Neuroblastoma can arise anywhere along the sympathetic nervous system. Fifty percent of the tumors originate in the adrenal medulla. Additional sites of origin include the nerve tissue in the chest, neck, pelvis or abdomen (Ries et al., 2005). Clinical presentation of neuroblastoma is highly variable and is dependent on the site of the primary tumor, as well as on the disease extent and on the absence or presence of paraneoplastic syndromes (Maris & Matthay, 1999; Park et al., 2008). The three main clinical scenarios are: localized, metastatic and 4S (S for special). In contrast to many other pediatric malignancies, progress in treatment of neuroblastoma (especially for advanced-stage tumors) has been relatively modest. Hence, at present, this tumor still poses a major challenge to the pediatric oncologist. Current treatment strategies include any or all of the following; watchfulwaiting, surgery, mild to aggressive chemotherapy, radiotherapy, and bone marrow transplants. Several new agents and combinations are in ongoing trials for relapsed neuroblastoma, including topoisomerase 1 inhibitors, radionuclides, histone deacetylase and tyrosine kinase inhibitors, monoclonal antibodies directed to disialoganglioside, and angiogenesis inhibitors (Maris & Matthay, 1999). As neuroblastoma is a disease that most often strikes young children, treating patients with aggressive therapy is a concern because of the potential for long-term health implications (from heart disease to second malignancies). Some children with neuroblastoma can be cured, and for these children, oncologists must try to give the minimum treatment possible while achieving cure. Although a fraction of the patients are cured with current treatments, approximately 40 per cent will die of this disease; for these patients improved treatment options are imperative (Matthay et al., 1999, 2009).
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2012
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Unknown venue
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2012-02-08
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Biology, Medicine
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