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RAF Fusion Genes and MAPK Activation in Pilocytic Astrocytomas

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Tumors of the Central Nervous System, Volume 5

Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 5))

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Abstract

Pilocytic astrocytomas (PA) are the most common childhood tumor of the central nervous system (CNS). Recent research has highlighted a key role for the mitogen activated protein kinase (MAPK) pathway in the development of these tumors. Whilst several mechanisms of activation of this pathway have been described in PA, it is most commonly caused by the formation of a BRAF fusion gene that encodes a protein with a constitutively active kinase domain. This fusion gene is highly specific to PAs when compared to other brain tumour subtypes, with direct implications for improved diagnostic procedures. Furthermore, the discovery of such a frequent molecular change underlying the development of these tumors affords a potential opportunity for targeted therapy of PAs.

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Authors and Affiliations

  1. Department of Pathology, University of Cambridge, Addenbrooke’s Hospital Box 231, Cambridge, CB2 0QQ, UK

    Sally R. Lambert

  2. Molecular Genetics of Pediatric Brain Tumors (B062), German Cancer Research Centre (DKFZ), Im Neuenheimer Feld 280, D-69120, Heidelberg, Germany

    David T. W. Jones

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  1. Sally R. Lambert
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  2. David T. W. Jones
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Correspondence to Sally R. Lambert .

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  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

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Lambert, S.R., Jones, D.T.W. (2012). RAF Fusion Genes and MAPK Activation in Pilocytic Astrocytomas. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 5. Tumors of the Central Nervous System, vol 5. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-2019-0_12

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  • DOI: https://doi.org/10.1007/978-94-007-2019-0_12

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  • Online ISBN: 978-94-007-2019-0

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