Ganglioneuroma pathophysiology
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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]
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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.