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- Title
Schwannomas and Their Pathogenesis.
- Authors
Hilton, David A.; Hanemann, Clemens Oliver
- 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 NF 2 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.
- Subjects
ACOUSTIC neuroma; NEUROFIBROMATOSIS 2; WORLD Health Organization; TUMORS; CHROMOSOMES; CALCIFICATION
- Publication
Brain Pathology, 2014, Vol 24, Issue 3, p205
- ISSN
1015-6305
- Publication type
Article
- DOI
10.1111/bpa.12125