Prognosis of neuroblastoma in childhood. Methods of assessment and clinical use

Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
Y PérelH Rubie

Abstract

Neuroblastoma and its benign counterpart, ganglioneuroma, are pediatric neuroblastic tumors arising in the sympathetic nervous system from neural-crest cells. Neuroblastoma, the most common extra-cranial solid tumour during childhood, is unique for its broad spectrum of clinical virulence from spontaneous remission to rapid and fatal progression despite intensive multimodality therapy. To a large extent, outcome could be predicted by the stage of disease and the age at diagnosis. However, a number of molecular events in neuroblastoma tumors, accounting for the variability of outcome and response to therapy, have been identified over the past decades. Among these, MYCN amplification is the most relevant prognostic factor and was the first genetic marker, in paediatric oncology, to be included in clinical strategies as a guide for therapeutic decision. This has allowed the most suitable intensity of therapy to be delivered according to a risk-stratified strategy, from observation to megadose chemotherapy with stem cell transplantation. Recent advances in understanding the biology and genetics of neuroblastoma will ultimately allow to select poor-risk patients for appropriate future biologically based therapies.

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Citations

Apr 8, 2010·Journal of Pediatric and Adolescent Gynecology·Joël MuhlsteinFanny Fouyssac
Apr 13, 2006·Pediatric Blood & Cancer·Patricia de CremouxJean Michon
Dec 29, 2009·Journal of Neurology·Aristidis DiamantisEmmanouil Magiorkinis
Feb 8, 2006·Journal of Pediatric Hematology/oncology·Ivy Zortéa S PariseBonald C Figueiredo

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