Clinical presentation, immunohistochemistry and electron microscopy indicate neurofibromatosis type 2-associated gliomas to be spinal ependymomas

Christian Hagel, Anat O. Stemmer-Rachamimov, Antje Bornemann, Martin Schuhmann, Christoph Nagel, Susan Huson, D. Gareth Evans, Scott Plotkin, Cordula Matthies, Lan Kluwe, Victor Felix Mautner

    Research output: Contribution to journalArticlepeer-review

    Abstract

    Neurofibromatosis type 2 (NF2) is a hereditary tumor syndrome. The hallmark of NF2 is bilateral vestibular schwannoma. In addition, glioma is one of the diagnostic criteria of NF2. In this retrospective study the clinical presentation and histopathological features of 12 spinal gliomas from NF2 patients were assessed. Ten tumors were previously diagnosed as ependymomas and two as astrocytomas. However, upon re-evaluation both astrocytomas expressed epithelial membrane antigen in a dot-like fashion and in one case it was possible to perform electron microscopy revealing junctional complexes and cilia typical for ependymoma. The findings suggest that NF2-associated spinal gliomas are ependymomas. Based on the fact that NF2-associated gliomas are almost exclusively spinal and that no NF2 mutations have been found in sporadic cerebral gliomas, we suggest that "glioma" in the current diagnostic criteria for NF2 should be specified as "spinal ependymoma". © 2012 Japanese Society of Neuropathology.
    Original languageEnglish
    Pages (from-to)611-616
    Number of pages5
    JournalNeuropathology
    Volume32
    Issue number6
    DOIs
    Publication statusPublished - Dec 2012

    Keywords

    • Electron microscopy
    • Ependymoma
    • Glioma
    • Immunohistochmistry
    • Neurofibromatosis type 2

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