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Published in: Molecular Diagnosis & Therapy 2/2013

01-04-2013 | Review Article

New Insights into the Genetics of Neuroblastoma

Implications for Diagnosis and Therapy

Authors: Srishma Sridhar, Batool Al-Moallem, Hawra Kamal, Marta Terrile, Raymond L. Stallings

Published in: Molecular Diagnosis & Therapy | Issue 2/2013

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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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Metadata
Title
New Insights into the Genetics of Neuroblastoma
Implications for Diagnosis and Therapy
Authors
Srishma Sridhar
Batool Al-Moallem
Hawra Kamal
Marta Terrile
Raymond L. Stallings
Publication date
01-04-2013
Publisher
Springer International Publishing AG
Published in
Molecular Diagnosis & Therapy / Issue 2/2013
Print ISSN: 1177-1062
Electronic ISSN: 1179-2000
DOI
https://doi.org/10.1007/s40291-013-0019-6

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