Ganglioglioma

Sarah Lapointe MD (Dr. Lapointe of the University of California, San Francisco, has no relevant financial relationships to disclose.)
Nicholas Butowski MD (Dr. Butowski of the University of California, San Francisco, has no relevant financial relationships to disclose.)
Edward J Dropcho MD, editor. (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released September 20, 1996; last updated November 12, 2017; expires November 12, 2020

This article includes discussion of ganglioglioma, gangliocytoma, ganglioglioneuroma, ganglion cell tumor, ganglioneuroglioma, ganglioneuroma, and neuroastrocytoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Ganglioglioma is an uncommon, usually low-grade, central nervous system (CNS) 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.

 

• Twenty to 60% of gangliogliomas have BRAF V600E mutation, and 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 (Perkins 1926). 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 characterize gangliogliomas.

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