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New Insights into the Genetics of Neuroblastoma

Implications for Diagnosis and Therapy

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Abstract

Neuroblastoma is a genetically and clinically heterogeneous tumor of childhood, arising from precursor cells of the sympathetic nervous system. It is still a challenging cancer for pediatric oncology, as some tumors will spontaneously regress, while others will become refractory to all forms of therapy. The clinical course of this disease is greatly influenced by both patient age and the genetic abnormalities that occur within the tumors. MYCN (v-myc myelocytomatosis viral related oncogene, neuroblastoma derived (avian)) amplification and loss of chromosome 11q heterozygosity have been known to be indicative of poor prognosis. In this article, we review how mutations and structural alterations in specific genes contribute to inheritable predisposition to neuroblastoma and/or to aggressive disease pathogenesis, as well as implications for diagnosis and therapy. These genes include PHOX2B (paired-like homeobox 2b), ALK (anaplastic lymphoma receptor tyrosine kinase), and ATRX (alpha thalassemia/mental retardation syndrome X-linked).

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Acknowledgments

This work was supported by grants from the Science Foundation Ireland (grant no. 07/IN.1/B1776), Children’s Medical and Research Foundation, Cancer Research Ireland, and US National Institutes of Health (grant no. 5R01CA127496). The authors have no conflicts of interest that are directly relevant to the content of this article.

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Authors and Affiliations

  1. School of Medicine, Royal College of Surgeons in Ireland, Bahrain, Manama, Bahrain

    Srishma Sridhar, Batool Al-Moallem & Hawra Kamal

  2. Department of Molecular and Cellular Therapeutics, Royal College of Surgeons in Ireland, York House, York Street, Dublin 2, Ireland

    Marta Terrile & Raymond L. Stallings

  3. National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin, Ireland

    Marta Terrile & Raymond L. Stallings

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  1. Srishma Sridhar
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Marta Terrile and Raymond L. Stallings are joint senior authors of this article.

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Sridhar, S., Al-Moallem, B., Kamal, H. et al. New Insights into the Genetics of Neuroblastoma. Mol Diagn Ther 17, 63–69 (2013). https://doi.org/10.1007/s40291-013-0019-6

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