Sign Up for a Free Account
  • Updated 10.03.2020
  • Released 09.20.1996
  • Expires For CME 10.03.2023

Ganglioglioma

Introduction

Overview

Ganglioglioma is an uncommon, usually low-grade, central nervous system tumor with 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.

• They 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.

• Though mostly low grade, World Health Organization (WHO) grade I, 5% are classified anaplastic ganglioglioma (WHO III or high grade). No criteria for WHO II gangliogliomas have been established.

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

• Identification of IDH1/2, TP53, or PTEN mutations or of CDK4 or EGFR amplification strongly argues against the diagnosis of ganglioglioma.

• Gangliogliomas are characterized by mitogen-activated protein kinase (MAPK) pathway activation through different genetic alterations, most commonly BRAF V600E mutation (20%–80% of gangliogliomas). BRAF V600E status should be determined for possible targeted therapy in case of residual and/or recurrent tumor.

Historical note and terminology

Gangliogliomas and gangliocytomas comprise a spectrum of low-grade tumors characterized by a 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 described in 1926 (29). Diagnosis was initially based on gross histopathology and light microcopy with additional use of electron microscopy. Histochemistry and immunocytoreactivity are currently used to demonstrate the ganglion cells component. The oncofetal marker CD34 and detection of BRAF V600E mutation or other MAPK pathway alterations characterize gangliogliomas.

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, 
including video clips of 
neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of 
neurology in 1,200 
comprehensive articles.

Questions or Comment?

MedLink, LLC

10393 San Diego Mission Rd, Suite 120

San Diego, CA 92108-2134

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com