Ganglioglioma pathophysiology: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Ganglioglioma}} | {{Ganglioglioma}} | ||
{{CMG}}{{AE}}{{ | {{CMG}}{{AE}}{{MC}} | ||
==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. <ref name="pmid23435618">{{cite journal| author=Koelsche C, Wöhrer A, Jeibmann A, Schittenhelm J, Schindler G, Preusser M et al.| title=Mutant BRAF V600E protein in ganglioglioma is predominantly expressed by neuronal tumor cells. | journal=Acta Neuropathol | year= 2013 | volume= 125 | issue= 6 | pages= 891-900 | pmid=23435618 | doi=10.1007/s00401-013-1100-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23435618 }} </ref> <ref name="pmid23609006">{{cite journal| author=Dahiya S, Haydon DH, Alvarado D, Gurnett CA, Gutmann DH, Leonard JR| title=BRAF(V600E) mutation is a negative prognosticator in pediatric ganglioglioma. | journal=Acta Neuropathol | year= 2013 | volume= 125 | issue= 6 | pages= 901-10 | pmid=23609006 | doi=10.1007/s00401-013-1120-y | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23609006 }} </ref> | |||
===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=== | ===Gross Pathology=== | ||
===Microscopic Pathology=== | ===Microscopic Pathology=== | ||
Gangliogliomas are composed of two cell populations:<ref name=dd>Pathophysiology of ganglioglioma. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioglioma</ref> | Gangliogliomas are composed of two cell populations:<ref name=dd>Pathophysiology of ganglioglioma. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioglioma</ref> | ||
*Ganglion cells (large mature neuronal elements): ''ganglio-'' | *Ganglion cells (large mature neuronal elements): ''ganglio-'' | ||
*Neoplastic glial elements ( | *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=== | ===Markers=== | ||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mandana Chitsazan, M.D. [2]
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
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
- ↑ 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.
- ↑ 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.
- ↑ Pathophysiology of ganglioglioma. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioglioma