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Low-grade epilepsy-associated neuroepithelial tumours — the 2016 WHO classification

Nature Reviews Neurology volume 12, pages 732–740 (2016)Cite this article

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Abstract

Rapid developments in molecular genetic technology and research have swiftly advanced our understanding of neuro-oncology. As a consequence, the WHO invited their expert panels to revise the current classification system of brain tumours and to introduce, for the first time, a molecular genetic approach for selected tumour entities, thus setting a new gold standard in histopathology. The revised 5th edition of the 'blue book' was released in May 2016 and will have a major impact in stratifying diagnosis and treatment. However, low-grade neuroepithelial tumours that present with early-onset focal epilepsy and are mostly seen in children and young adults (previously designated as long-term epilepsy-associated neuroepithelial tumours, LEAT) lack such innovative clinicopathological and molecular genetic tools. The Neuropathology Task Force of the International League against Epilepsy will critically discuss this issue, and will offer perspectives on how to decipher and validate clinically meaningful LEAT entities using the current WHO approach that integrates clinicopathological and genetic classification systems.

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Figure 1: Is it all in one? The histopathological spectrum of nodular growth in low-grade epilepsy-associated neuroepithelial tumours.
Figure 2: Molecular genetic pathways affected in low-grade epilepsy-associated neuroepithelial tumours.
Figure 3: Roadmap to comprehensive clinicopathological and genetic WHO–ILAE classification of epilepsy-associated neuroepithelial tumours.

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Acknowledgements

Members of the ILAE Neuropathology Task Force have been partially supported by the Seventh Framework Programme of the European Commission (DESIRE GA # 602531 to I.B.; EPITARGET GA # 602102 to A.B., and E.A.; EPISTOP # 602391 to E.A.). E.A. has received funding from KIKA (Stichting Kinderen Kankervrij) and Stichting AMC Foundation. T.J. has received funding from the Brain Tumour Charity, Children with Cancer, Great Ormond Street Children's Charity and NIHR. H.M. has received funding from the Japan Epilepsy Research Foundation (JERF) (H25-003).

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Authors and Affiliations

  1. Roland Coras and Katja Kobow are at the Department of Neuropathology, Ingmar Blümcke, University Hospital Erlangen, Schwabachanlage 6, D-91054 Erlangen, Germany.,

    Ingmar Blümcke, Roland Coras & Katja Kobow

  2. Eleonora Aronica and Angelika Mühlebner are at the Department of NeuroPathology, Academic Medisch Centrum, Meibergdreef 9, 1105AZ Amsterdam, Netherlands.,

    Eleonora Aronica & Angelika Mühlebner

  3. Albert Becker is at the Department of Neuropathology, University of Bonn Medical Centre, Sigmund-Freud Str. 25, D – 53105 Bonn, Germany.,

    Albert Becker

  4. David Capper is at the Department of Neuropathology, Institute of Pathology, Im Neuenheimer Feld 224, 69120 Heidelberg, Ruprecht-Karls-University Heidelberg, Heidelberg, Germany.,

    David Capper

  5. Mrinalini Honavar is at the Department of Anatomic Pathology, Hospital Pedro Hispano, Rua Dr Eduardo Torres, 4460–504 Matosinhos, Portugal.,

    Mrinalini Honavar

  6. Thomas S. Jacques is at the Developmental Biology and Cancer Programme, UCL Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK.,

    Thomas S. Jacques

  7. Hajime Miyata is at the Department of Neuropathology, Research Institute for Brain and Blood Vessels – AKITA, 6–10 Senshu-Kubota-Machi, Akita City, Akita 010–0874, Japan.,

    Hajime Miyata

  8. Jose Pimentel is at the Laboratory of Neuropathology, Hospital de Santa Maria, (CHLN), Av. Prof. Egas Moniz, 1649–036, Lisbon, Portugal.,

    José Pimentel

  9. Figen Söylemezog˘lu is at the Department of Pathology, Hacettepe University Faculty of Medicine, Sihhiye, Ankara, TR-06100 Turkey.,

    Figen Söylemezoğlu

  10. Maria Thom is at the Department of Neuropathology, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK.,

    Maria Thom

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Blümcke, I., Aronica, E., Becker, A. et al. Low-grade epilepsy-associated neuroepithelial tumours — the 2016 WHO classification. Nat Rev Neurol 12, 732–740 (2016). https://doi.org/10.1038/nrneurol.2016.173

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