Molecular biology of neuroblastoma

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Klin Onkol 2005; 18(5): 159-165.

Summary:Neuroblastoma is a malignant embryonal childhood tumor originating from immature and undifferentiated neural crest-derived cells which colonize paravertebral sympathetic ganglia, adrenal medulla and paraganglia. The clinical hallmark of neuroblastoma is heterogeneity. Low-risk tumors undergo spontaneous regression or spontaneousor therapy-induced differentiation. There is characteristic aggressive progression with fast growth and early metastases in high-risk tumors. Several parameters have been proposed to predict biological behavior: age at the time of diagnosis, histological classification, expression of trk receptors, DNA ploidy, N-myc amplification, 1p36 deletion and many other deletions and gains (2q, 3p, 9p, 11q, 13q, and 14q deletion and 17q gain). We can demonstrate the importance of disease course prediction and risk adapted therapy using the neuroblastoma model. On the other hand, it is also possible to show correlations of many prognostic factors from which only some bring independent information. Multifactorial analyses of data from a large multicentric study are necessary for evaluation of prognostic significance of a complex of factors.

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