Neuroblastoma – telomere maintenance, deregulated signalling transduction and beyond

Cet article passe en revue les différents sous-types moléculaires des neuroblastomes, analyse l'influence de ces sous-types moléculaires sur la stratification du risque, décrit les avancées récentes en matière de thérapies ciblées et examine le rôle des mécanismes d'entretien des télomères dans la progression tumorale

Résumé en anglais

Abstract The childhood malignancy neuroblastoma belongs to the group of embryonal tumours and originates from progenitor cells of the sympathoadrenal lineage. Treatment options for children with high-risk and relapsed disease are still very limited. In recent years, an ever-growing molecular diversity was identified using (epi)-genetic profiling of neuroblastoma tumours, indicating that molecularly targeted therapies could be a promising therapeutic option. In this review article, we summarize the various molecular subtypes and genetic events associated with neuroblastoma and describe recent advances in targeted therapies. We lay a strong emphasis on the importance of telomere maintenance mechanisms for understanding tumour progression and risk classification of neuroblastoma. This article is protected by copyright. All rights reserved.