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Gangliogliomas: Molecular Pathogenesis and Epileptogenesis

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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

Gangliogliomas (GG) constitute the most frequent tumor entity in young patients undergoing surgery for intractable epilepsy. Surgery provides the best chance for curing epilepsy and preventing malignant transformation. GG consist of a mixture of dysplastic neurons and neoplastic astroglial cells. Although malignant transformation of GG is rare, it has been documented in their astroglial component. The characteristic histopathological features of GG together with the coexistence with cortical dysplasia and the expression of stem cell markers (such as CD34) suggest a developmental origin for these lesions. The malformative nature of GG is also supported by the detection of molecular alterations common to other developmental glioneuronal lesions. In particular, recent studies suggest a role for the phosphatidyl-inositol 3-kinase (PI3K)-mTOR pathway in the molecular pathogenesis of glioneuronal lesions. The cellular mechanism(s) underlying the epileptogenicity of GG is still not clearly defined. Several mechanisms are possibly involved. Recent studies support the role of alterations of the balance between excitation and inhibition, and neuron-glia interactions may also play a critical role in the generation of seizures. Astroglial cells express functional receptors for a variety of neurotransmitters and may critically modulate synaptic transmission. In addition, an increasing number of observations indicate that specific inflammatory pathways are activated in GG and may contribute to the onset of seizures and epileptogenesis.

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Acknowledgments

We thank Dr. Albert Becker (Dept. of Neuropathology, University of Bonn Medical Center) for his input in the final version of this chapter.

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Authors and Affiliations

  1. Department of Neuropathology, Academic Medical Center, 1105 AZ, Amsterdam, The Netherlands

    Eleonora Aronica

  2. Department of Neuropathology, University of Bonn, Medical Center, Sigmund-Freud-Str. 25, 53105, Bonn, Germany

    Pitt Niehusmann

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  1. Eleonora Aronica
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Correspondence to Eleonora Aronica .

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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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Aronica, E., Niehusmann, P. (2012). Gangliogliomas: Molecular Pathogenesis and Epileptogenesis. 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_29

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  • DOI: https://doi.org/10.1007/978-94-007-2019-0_29

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  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-007-2018-3

  • Online ISBN: 978-94-007-2019-0

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