Abstract
Neuroblastoma is a highly malignant tumor of infants and children. It typically occurs before the age of 5 years and accounts for up to 50% of all malignancies among infants [for review see 1,2]. A significant fraction of cases are identified neonatally, indicating that the tumor can arise during fetal life and may represent a disorder of normal development [3]. These tumors arise in sympathetic neuroblasts that originate in the neural crest and are destined to become chromaffin or neuronal tissues of the peripheral nervous system [4]. Sixty-five percent of neuroblastomas occur in the abdomen, where adrenal medullary tumors account for 40% of these tumors [1,2]. Approximately 50% of infants and 70% of older children with neuroblastomas have evidence of tumor spread beyond the primary location to metastatic sites, including the lymph nodes, bone, bone marrow, liver, and skin, at the time they first come to medical attention [1]. In children under 1 year of age, a special presentation of disseminated neuroblastoma, which is most clearly distinguished from the more common presentation of advanced-stage neuroblastoma in that it does not involve lytic lesions of the bone, has been recognized [5–8]. This group, designated stage IVs, includes approximately 17% of neuroblastoma tumors arising in children under the age of 1 year. Remarkably these tumors regress without therapy, while those of older patients or of young children with metastatic disease to bone have a very poor prognosis [1].
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El-Badry, O.M., Israel, M.A. (1993). Growth regulation of human neuroblastoma. In: Benz, C.C., Liu, E.T. (eds) Oncogenes and Tumor Suppressor Genes in Human Malignancies. Cancer Treatment and Research, vol 63. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-3088-6_5
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