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  • Updated 09.11.2023
  • Released 09.20.1996
  • Expires For CME 09.11.2026




Ganglioglioma is an uncommon, usually low-grade, central nervous system tumor composed of cells resembling neuronal tissue and glial cells. In this article, the authors discuss diagnosis and treatment of ganglioglioma.

Key points

• Gangliogliomas are well-differentiated, slow-growing, mixed neuronal-glial tumors composed of dysplastic ganglion cells (neurons) and neoplastic glial cells.

• Gangliogliomas should be suspected in any child or young adult with temporal-lobe epilepsy and a temporal lobe cortical-based lesion on imaging, although these tumors can be found throughout the central nervous system.

• No formal criteria for WHO grade 2 gangliogliomas have been established.

• Gross total resection of the tumor remains the only effective treatment in most cases, and prognosis is excellent when a WHO grade 1 ganglioglioma is grossly, totally resected.

Historical note and terminology

Gangliogliomas and gangliocytomas comprise a spectrum of low-grade tumors characterized by dysplastic neuronal (or ganglion) cell types. In gangliogliomas, the dysplastic neuronal cells are accompanied by neoplastic glial cells, whereas in gangliocytomas, large, multipolar binucleated well-differentiated neurons are the unique neoplastic component.

The term ganglioglioma was originally coined in 1926 (38). The diagnosis was initially based on gross histopathology and light microscopy with additional use of electron microscopy. Histochemistry and immunocytoreactivity are used to demonstrate the ganglion cells component. The oncofetal marker CD34 and detection of BRAF V600E mutation or other MAPK pathway alterations characterize gangliogliomas.

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