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

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: particularly with mucosal ganglioneuromas.^[1]
Turner syndrome
Neurofibromatosis type 1

Gross Pathology

Ganglioneuromas are solid, firm tumors that are typically white when seen with the naked eye
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.
They are composed of ganglion cells, schwann cells and fibrous tissue.
Histopathology 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.

Template:WikiDoc Sources

[ddd-1] 1.0 ^1.1 Pathology of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma

[pmid22907039-2] 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.

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