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The role of genetic and epigenetic alterations in neuroblastoma disease pathogenesis.

ABSTRACT: Neuroblastoma is a highly heterogeneous tumor accounting for 15 % of all pediatric cancer deaths. Clinical behavior ranges from the spontaneous regression of localized, asymptomatic tumors, as well as metastasized tumors in infants, to rapid progression and resistance to therapy. Genomic amplification of the MYCN oncogene has been used to predict outcome in neuroblastoma for over 30 years, however, recent methodological advances including miRNA and mRNA profiling, comparative genomic hybridization (array-CGH), and whole-genome sequencing have enabled the detailed analysis of the neuroblastoma genome, leading to the identification of new prognostic markers and better patient stratification. In this review, we will describe the main genetic factors responsible for these diverse clinical phenotypes in neuroblastoma, the chronology of their discovery, and the impact on patient prognosis.

SUBMITTER: Domingo-Fernandez R

PROVIDER: S-EPMC3557462 | biostudies-literature | 2013 Feb

REPOSITORIES: biostudies-literature

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The role of genetic and epigenetic alterations in neuroblastoma disease pathogenesis.

Domingo-Fernandez Raquel R Watters Karen K Piskareva Olga O Stallings Raymond L RL Bray Isabella I

Pediatric surgery international 20121229 2

Neuroblastoma is a highly heterogeneous tumor accounting for 15 % of all pediatric cancer deaths. Clinical behavior ranges from the spontaneous regression of localized, asymptomatic tumors, as well as metastasized tumors in infants, to rapid progression and resistance to therapy. Genomic amplification of the MYCN oncogene has been used to predict outcome in neuroblastoma for over 30 years, however, recent methodological advances including miRNA and mRNA profiling, comparative genomic hybridizati ...[more]

PMID: 23274701

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