Overview

NCI Definition: A well differentiated, slow growing neuroepithelial neoplasm composed of neoplastic, mature ganglion cells and neoplastic glial cells. Some gangliogliomas show anaplastic features in their glial component and are considered to be WHO grade III. Rare cases of newly diagnosed gangliogliomas with grade IV (glioblastoma) changes in the glial component have also been reported. (Adapted from WHO) [1]

Gangliogliomas most frequently harbor alterations in BRAF, ZFHX3, NF1, KDR, and ARID1B [2].

Most Commonly Altered Genes in Ganglioglioma

BRAF Mutation, BRAF Exon 15 Mutation, BRAF V600E, BRAF Codon 600 Missense, and KDR Mutation are the most common alterations in ganglioglioma [2].

Top Alterations in Ganglioglioma

Biomarker-Directed Therapies

Significant Genes in Ganglioglioma

ARAF +

BRAF +

CRKL +

FGFR1 +

FGFR2 +

FGFR3 +

HRAS +

KRAS +

MAP2K1 +

MAP2K2 +

MAP2K4 +

MAP3K1 +

MAPK1 +

MYB +

MYBL1 +

NF1 +

NF2 +

NRAS +

PTPN1 +

RAF1 +

SOS1 +

SRC +

Disease Details

Synonyms
GANGLIOGLIOMA, UNDETERMINED
Parent(s)
Neuronal and Mixed Neuronal-Glial Tumors
OncoTree Name
Ganglioglioma
OncoTree Code
GNG

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.