Ganglioneuroma pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
On gross pathology, ganglioneuromas are characterized by solid, white, firm, well-circumscribed and nodular tumors. | On gross pathology, ganglioneuromas are characterized by solid, white, firm, well-circumscribed, and nodular tumors. | ||
==Pathophysiology== | ==Pathophysiology== | ||
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===Gross Pathology=== | ===Gross Pathology=== | ||
*Ganglioneuromas are solid, white, firm, well-circumscribed and nodular tumors. | *Ganglioneuromas are solid, white, firm, well-circumscribed, and nodular tumors. | ||
*Gangliocytoma is commonly located in the following regions:<ref name=ddd>Pathology of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma</ref> | *Gangliocytoma is commonly located in the following regions:<ref name=ddd>Pathology of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma</ref> | ||
**Posterior [[paraspinal mediastinum]] (most common) | **Posterior [[paraspinal mediastinum]] (most common) | ||
Revision as of 14:16, 11 September 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
On gross pathology, ganglioneuromas are characterized by solid, white, firm, well-circumscribed, and nodular tumors.
Pathophysiology
Genetics
Development of ganglioneuroma is the result of multiple genetic mutations. Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36.
Associated Conditions
- Multiple endocrine neoplasia type IIb (mucosal ganglioneuromas).[1]
- Turner syndrome
- Neurofibromatosis type 1
Gross Pathology
- Ganglioneuromas are solid, white, firm, well-circumscribed, and nodular tumors.
- Gangliocytoma is commonly located in the following regions:[1]
- Posterior paraspinal mediastinum (most common)
- Adrenal gland
- Paraspinal retroperitoneum
- Neck
Microscopic Pathology
- Ganglioneuromas are derived from the primordial neural crest cells, which are undifferentiated cells of the sympathetic nervous system.
- Ganglioneuromas are fully differentiated neuronal tumors that do not contain immature elements.
- Histopathologically, ganglioneuroma consists of spindled cells, with cell borders in a fibrillar matrix which contain ganglion cells with large round nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. No atypia or mitotic activity was evident. The ganglion cells stained positive for S-100 protein.[2]
Videos
{{#ev:youtube|IWng6E9flDA}}
References
- ↑ 1.0 1.1 Pathology of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma
- ↑ Vasiliadis K, Papavasiliou C, Fachiridis D, Pervana S, Michaelides M, Kiranou M; et al. (2012). "Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: A case report". Int J Surg Case Rep. 3 (11): 541–3. doi:10.1016/j.ijscr.2012.07.008. PMC 3437388. PMID 22907039.