Nervous system: Embryonal tumors: Neuroblastoma
Open Access
- 1 July 2020
- journal article
- Published by INIST-CNRS in Atlas of Genetics and Cytogenetics in Oncology and Haematology
- Vol. 24 (7), 284-290
- https://doi.org/10.4267/2042/70771
Abstract
Neuroblastoma is a clinically heterogenous pediatric cancer of the sympathetic nervous system that originates from neural crest cells. It is the most common extracranial solid tumor in childhood and prognosis ranges from spontaneous tumor regression to aggressive disease resistant to multimodal therapy. Prognosis depends on patient characteristics and tumor biology that determine risk classification. Advancements in therapy reductions are merited for low- and intermediate-risk neuroblastoma patients, who generally have excellent outcomes. Of the patients with high-risk disease, only 50% achieve long-term survival, and therapeutic advancements are needed. Over the past several decades, genomic features such as germline mutations, somatic genetic aberrations, chromosome copy number, transcriptomics, and epigenetics have proven to contribute to the pathogenesis of neuroblastoma. The primary predisposition genes in familial neuroblastoma are ALK and PHOX2B. Sporadic neuroblastoma arises with complex pathogenesis, but chromosomal abnormalities and single-nucleotide polymorphisms have been identified to cooperatively lead to oncogenesis. These advances have led to new therapeutic approaches with the potential to improve outcomes for children with neuroblastoma.Keywords
This publication has 29 references indexed in Scilit:
- Segmental chromosomal alterations have prognostic impact in neuroblastoma: a report from the INRG projectBritish Journal of Cancer, 2012
- Common variation at 6q16 within HACE1 and LIN28B influences susceptibility to neuroblastomaNature Genetics, 2012
- Phenotype Restricted Genome-Wide Association Study Using a Gene-Centric Approach Identifies Three Low-Risk Neuroblastoma Susceptibility LociPLoS Genetics, 2011
- Integrative genomics identifies LMO1 as a neuroblastoma oncogeneNature, 2010
- Anti-GD2 Antibody with GM-CSF, Interleukin-2, and Isotretinoin for NeuroblastomaThe New England Journal of Medicine, 2010
- Common variations in BARD1 influence susceptibility to high-risk neuroblastomaNature Genetics, 2009
- The International Neuroblastoma Risk Group (INRG) Classification System: An INRG Task Force ReportJournal of Clinical Oncology, 2009
- Identification of ALK as a major familial neuroblastoma predisposition geneNature, 2008
- The kinesin KIF1Bβ acts downstream from EglN3 to induce apoptosis and is a potential 1p36 tumor suppressorGenes & Development, 2008
- Germline Mutations of the Paired–Like Homeobox 2B (PHOX2B) Gene in NeuroblastomaAmerican Journal of Human Genetics, 2004