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Neuroblastoma

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neuroblastoma

(Science: oncology, tumour) malignant tumour derived from primitive ganglion cells. Mainly a tumour of childhood. Commonest sites are adrenal medulla and retroperitoneal tissue. The cells may partially differentiate into cells having the appearance of immature neurons.

The tumour shows a consistent deletion of the 1p36 region which is considered the site of the neuroblastoma I gene. Loss of the 1p36 region also correlates with amplification for the N-myc oncogene, particularly in later stages of the disease.

amplification of N-myc with dNA triploidy is associated with poor survival.

hereditary forms of the condition are rare and in such patients there is no risk for other cancers, which suggests a specificity of the antioncogene for neural tissues.