neuroblastomamedical dictionary

<tumour>

<oncology> 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 neurones.

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.

(16 Dec 1997)

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