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BMC Cancer
Published in: BMC Cancer 1/2017

Open Access 01-12-2017 | Debate

Growth, progression and chromosome instability of Neuroblastoma: a new scenario of tumorigenesis?

Author: Gian Paolo Tonini

Published in: BMC Cancer | Issue 1/2017

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Abstract

Background

Neuroblastoma is a pediatric cancer with a low survival rate of patients with metastatic stage 4 disease. Tumor aggressiveness and progression have been associated with structural copy number variations (CNVs) that are observed in malignant cells. In contrast, localized Neuroblastomas, which are associated with a low number of structural CNVs but frequent numerical CNVs, are less aggressive, and patients have good outcomes. Finally, whole-genome and whole-exome sequencing of Neuroblastoma tissues have shown few damaging mutations in these tumors.

Conclusions

In the present report it is proposed that chromosome instability (CIN) plays a major role in Neuroblastoma tumorigenesis and that CIN is already present in the early phases of tumor development. High CIN can promote several types of chromosomal damage including chromothripsis, gene deletion, amplification and rearrangements, which deregulate gene expression. Indeed, gene rearrangements have been reported as a new scenario in the development of Neuroblastoma, which supports the hypothesis that CIN is an early step preliminary to the late catastrophic events leading to tumor development.
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Metadata
Title
Growth, progression and chromosome instability of Neuroblastoma: a new scenario of tumorigenesis?
Author
Gian Paolo Tonini
Publication date
01-12-2017
Publisher
BioMed Central
Published in
BMC Cancer / Issue 1/2017
Electronic ISSN: 1471-2407
DOI
https://doi.org/10.1186/s12885-016-2986-6

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