Journal
INTERNATIONAL JOURNAL OF CANCER
Volume 135, Issue 10, Pages 2249-2261Publisher
WILEY-BLACKWELL
DOI: 10.1002/ijc.29077
Keywords
neuroblastoma; susceptibility; genetic alterations; expression signatures; progression; animal models
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Funding
- The Institut National du Cancer
- The Ligue Nationale contre le Cancer (Equipe labellisee)
- The Association Hubert Gouin
- Les Bagouz a Manon
- les amis de Claire
- la course de Timo
- Courir pour Mathieu
- Dans les pas du Geant
- Olivier Chape
- la Federation Enfants et Sante et la Societe Francaise de Lutte contre les Cancers et les Leucemies de l'Enfant et l'Adolescent
- The Annenberg Foundation
- The Siric/INCA [INCa-DGOS-4654]
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Neuroblastoma (NB) is an embryonal tumor of the sympathetic nervous system which accounts for 8-10% of pediatric cancers. It is characterized by a broad spectrum of clinical behaviors from spontaneous regression to fatal outcome despite aggressive therapies. Considerable progress has been made recently in the germline and somatic genetic characterization of patients and tumors. Indeed, predisposition genes that account for a significant proportion of familial and syndromic cases have been identified and genome-wide association studies have retrieved a number of susceptibility loci. In addition, genome-wide sequencing, copy-number and expression studies have been conducted on tumors and have detected important gene modifications, profiles and signatures that have strong implications for the therapeutic stratification of patients. The identification of major players in NB oncogenesis, including MYCN, ALK, PHOX2B and LIN28B, has enabled the development of new animal models. Our review focuses on these recent advances, on the insights they provide on the mechanisms involved in NB development and their applications for the clinical management of patients.
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