Ganglioneuroma pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Ganglioneuromas are derived from the primordial neural crest cells, which are undifferentiated cells of the sympathetic nervous system. Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36. 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.^[1]

• Ganglioneuromas are derived from the primordial neural crest cells, which are undifferentiated cells of the sympathetic nervous system.
• They are composed of ganglion cells, schwann cells, and fibrous tissue.^[2]
• They do not contain neuroblasts, intermediate cells, or mitotic figures.

Development of ganglioneuroma is the result of multiple genetic mutations. Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36.

Ganglioneuromas may be associated with:^[2]

• Multiple endocrine neoplasia type IIb (mucosal ganglioneuromas)
• Turner syndrome
• Neurofibromatosis type 1

• On gross pathology, ganglioneuromas are characterized by solid, white, firm, well-circumscribed, and nodular tumors.
• Gangliocytoma is commonly located in the following regions:^[2]
  • Posterior paraspinal mediastinum (most common)
  • Adrenal gland
  • Paraspinal retroperitoneum
  • Neck

• 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. No atypia or mitotic activity is evident. The ganglion cells stain positive for S-100 protein.^[1]
• Ganglioneuromas are well differentiated neuronal tumors that do not contain immature elements.

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