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Histology and Molecular Genetics

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Primary Optic Nerve Sheath Meningioma

Part of the book series: Medical Radiology ((Med Radiol Radiat Oncol))

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Abstract

Optic nerve sheath meningiomas (ONSM) are rare tumors of the anterior visual pathway constituting approximately 2% of all intraorbital tumors and about 1%–2% of meningiomas. They arise from the meningeal coverings of the central nervous system either as primary or secondary orbital lesions. Primary ONSM develop intraorbitally at any location along the optical nerve. They share the same histological features as meningiomas in other locations of the central nervous system and are histologically classified according to the ‘World Health Organization (WHO) Classification of Tumours of the Central Nervous System’. Most commonly presenting with a slow growth and a benign clinical behavior, most meningiomas correspond to WHO grade I. Meningothelial and transitional histological variants are the most frequent subtypes of ONSM. However, rare cases are associated with a higher risk of recurrence and shorter survival times. The WHO classification assigns these lesions to WHO grades II and III. This chapter provides a review on meningioma histology in general and about histopathological aspects of ONSM in particular. Moreover, it shortly outlines the underlying molecular alterations that initiate meningioma growth and promote meningioma progression.

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Authors and Affiliations

  1. Department of Neuropathology, Heinrich-Heine-University, Moorenstr. 5, 40225, Duesseldorf, Germany

    Markus J. Riemenschneider & Guido Reifenberger

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  1. Markus J. Riemenschneider
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  2. Guido Reifenberger
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  1. International Atomic Energy Agency, Wagramer Strasse 5, P.O. Box 100, 1400, Vienna, Austria

    Branislav Jeremic

  2. University Eye Hospital, Langenbeckstrasse 1, 55101, Mainz, Germany

    Susanne Pitz

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© 2008 Springer-Verlag Berlin Heidelberg

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Riemenschneider, M., Reifenberger, G. (2008). Histology and Molecular Genetics. In: Jeremic, B., Pitz, S. (eds) Primary Optic Nerve Sheath Meningioma. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-77558-4_5

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  • DOI: https://doi.org/10.1007/978-3-540-77558-4_5

  • Publisher Name: Springer, Berlin, Heidelberg

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