Ganglioneuroma overview

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Pathophysiology

Causes

Differentiating Ganglioneuroma from other Diseases

Epidemiology and Demographics

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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

Classification

According to International Neuroblastoma Pathology Classification (INPC), ganglioneuroma may be classified into two subtypes/groups: Ganglioneuroma-maturing and ganglioneuroma-mature.[1]

Pathophysiology

On gross pathology, ganglioneuromas are characterized by solid, white, firm, well-circumscribed, and nodular tumors. On microscopic histopathological analysis, ganglioneuromas are characterized by spindle-shaped cells with cell borders in a fibrillar matrix containing ganglion cells with large round nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm.[2] Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36.

Causes

There are no established causes for ganglioneuroma.[3]

Differentiating brain tumors from other diseases

Ganglioglioma must be differentiated from neuroblastoma, ganglioneuroblastoma, spinal schwannoma, spinal neurofibroma, adrenal adenoma, adrenal carcinoma, and pheochromocytoma.[3][4]

Epidemiology and Demographics

The incidence of ganglioneuroma is approximately 1 per 100,000 children in the United States.[4] Ganglioneuroma is a rare disease that tends to affect children and young adults.[3] Females are more commonly affected with ganglioneuroma than males.[4] There is no racial predilection to ganglioneuroma.

Risk factors

There are no established risk factors for ganglioneuroma.[3]

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical examination

Laboratory Findings

X Ray

CT

MRI

Ultrasound

Other imaging findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

References

  1. Okamatsu C, London WB, Naranjo A, Hogarty MD, Gastier-Foster JM, Look AT; et al. (2009). "Clinicopathological characteristics of ganglioneuroma and ganglioneuroblastoma: a report from the CCG and COG". Pediatr Blood Cancer. 53 (4): 563–9. doi:10.1002/pbc.22106. PMC 2730988. PMID 19530234.
  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.
  3. 3.0 3.1 3.2 3.3 Causes for ganglioneuroma. National Library of Meidicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
  4. 4.0 4.1 4.2 Adam, O; Boia, ES (2007). "ganglioneuroma" (PDF). jurnalul pediatrului. 10 (39–40). Retrieved 10 September 2015.


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