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Ganglioglioma pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mandana Chitsazan, M.D. [2]

Overview

Overview

A ganglioglioma arises from neuronal glial cells, which are cells of the central nervous system. On gross pathology, a ganglioglioma varies from being a partially cystic mass with a mural nodule to a solid mass expanding the overlying gyrus. On microscopic pathology, a ganglioglioma is composed of ganglion cells and neoplastic glial cells with positive staining for synaptophysin, neuronal specific enolase, and GFAP.

Pathophysiology

Pathophysiology

Genetics

Mutation of BRAF V600E gene has been detected in ganglioglioma. The mutant BRAF protein is expressed predominantly in neural cells and to a lesser degree, in the glial component. [1] [2]

Pathogenesis

Different pathways may be involved in the molecular pathogenesis of the ganglioglioma. Activation of the PI3K-Akt-mTOR pathway has been noted in ganglioglioma.


Gross Pathology

Microscopic Pathology

Gangliogliomas are composed of two cell populations:[3]

  • Ganglion cells (large mature neuronal elements): ganglio-
  • Neoplastic glial elements (astrocytic): -glioma

The glial component determines the biological behaviour of ganglioglioma. Dedifferentiation into high grade tumors may occur, and usually involves the glial component.

Markers

References

References

  1. Koelsche C, Wöhrer A, Jeibmann A, Schittenhelm J, Schindler G, Preusser M; et al. (2013). “Mutant BRAF V600E protein in ganglioglioma is predominantly expressed by neuronal tumor cells”. Acta Neuropathol. 125 (6): 891–900. doi:10.1007/s00401-013-1100-2. PMID 23435618.
  2. Dahiya S, Haydon DH, Alvarado D, Gurnett CA, Gutmann DH, Leonard JR (2013). “BRAF(V600E) mutation is a negative prognosticator in pediatric ganglioglioma”. Acta Neuropathol. 125 (6): 901–10. doi:10.1007/s00401-013-1120-y. PMID 23609006.
  3. Pathophysiology of ganglioglioma. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioglioma


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