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Neuroblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information

Fortunately, cancer in children and adolescents is rare, although the overall incidence of childhood cancer has been slowly increasing since 1975.[1] Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the primary care physician, pediatric surgical subspecialists, radiation oncologists, pediatric medical oncologists/hematologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others to ensure that children receive treatment, supportive care, and rehabilitation that will enable them to achieve optimal survival and quality of life. (Refer to the PDQ summaries on Supportive and Palliative Care for specific information about supportive care for children and adolescents with cancer).

Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics.[2] At these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate in these trials is offered to most patients and families. Clinical trials for children and adolescents with cancer are generally designed to compare potentially better therapy with therapy that is currently accepted as standard. Most of the progress made in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

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Dramatic improvements in survival have been achieved for children and adolescents with cancer.[1] Between 1975 and 2002, childhood cancer mortality has decreased by more than 50%. For neuroblastoma, the 5-year survival rate in the United States has remained stable at approximately 87% for children younger than 1 year and has increased from 37% to 65% in children aged 1 to 14 years.[1] Childhood and adolescent cancer survivors require close follow-up since cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors).

Neuroblastoma is predominantly a tumor of early childhood, with two-thirds of the cases presenting in children aged 5 years or younger. Neuroblastoma originates in the adrenal medulla or the paraspinal sites where sympathetic nervous system tissue is present. These tumors can be divided into low-, intermediate-, and high-risk groups as illustrated in the Stage Information section of this summary. Low- and intermediate-risk patients usually have localized disease or are infants aged 18 months or younger. In rare cases, neuroblastoma can be discovered prenatally by fetal ultrasonography.[3]

Predisposition to Neuroblastoma

Little is known about the events that predispose to the development of neuroblastoma. Parental exposures have not been definitively linked. In a genome-wide association study of 1,032 patients with neuroblastoma, a significant association was observed between a common genetic variation (polymorphism) at chromosome 6p22 and neuroblastoma. Tumors that arose in patients with this polymorphism tended to be clinically aggressive.[4] Genome-wide association studies in children with neuroblastoma have found a few gene polymorphisms associated with the development of high-risk neuroblastoma and a few other gene polymorphisms associated with the development of low-risk neuroblastoma.[4,5] Germline deletion at the 1p36 or 11q14-23 locus are associated with the development of neuroblastoma and the same deletions are found somatically in sporadic neuroblastomas.[6,7]


WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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