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      Schwannomas and their pathogenesis.

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          Abstract

          Schwannomas may occur spontaneously, or in the context of a familial tumor syndrome such as neurofibromatosis type 2 (NF2), schwannomatosis and Carney's complex. Schwannomas have a variety of morphological appearances, but they behave as World Health Organization (WHO) grade I tumors, and only very rarely undergo malignant transformation. Central to the pathogenesis of these tumors is loss of function of merlin, either by direct genetic change involving the NF2 gene on chromosome 22 or secondarily to merlin inactivation. The genetic pathways and morphological features of schwannomas associated with different genetic syndromes will be discussed. Merlin has multiple functions, including within the nucleus and at the cell membrane, and this review summarizes our current understanding of the mechanisms by which merlin loss is involved in schwannoma pathogenesis, highlighting potential areas for therapeutic intervention.

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          Author and article information

          Journal
          Brain Pathol.
          Brain pathology (Zurich, Switzerland)
          1750-3639
          1015-6305
          Apr 2014
          : 24
          : 3
          Affiliations
          [1 ] Department of Cellular and Anatomical Pathology, Derriford Hospital, Plymouth, UK.
          Article
          10.1111/bpa.12125
          24450866
          © 2014 International Society of Neuropathology.

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