Genetic parameters of neuroblastomas

作者: Frank Westermann , Manfred Schwab

DOI: 10.1016/S0304-3835(02)00199-4

关键词:

摘要: Neuroblastoma is a malignant childhood tumor of migrating neuroectodermal cells derived from the neural crest and destined for adrenal medulla sympathetic nervous system. The biological behavior neuroblastomas extremely variable in some respects unique. Neuroblastomas tend to regress spontaneously portion infants or differentiate into benign ganglioneuroma older patients. Unfortunately, majority patients neuroblastoma metastatic at time diagnosis, it usually undergoes rapid progression with fatal outcome. mechanisms leading this diverse clinical are largely unclear. From analysis tumors cytogenetic molecular level non-random genetic changes have been identified, including ploidy changes, amplification oncogene MYCN, deletions chromosome 1p, gains arm 17q, 11q as well other genomic regions that allow be classified subsets distinct features behavior. MYCN status widely accepted therapy stratification. Additional parameters currently under investigation refine risk assessment, but so far monitoring tools prediction response disease outcome still incomplete. This should lead more risk-adapted therapies according clinical-genetic by which individual characterized. review aims discussing role types.

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