Overview

NCI Definition: A group of central nervous system neoplasms with a variable amount of neuronal and, less consistently, glial differentiation. They occur at a low frequency and usually carry a favorable prognosis. Representative examples include dysplastic cerebellar gangliocytoma, desmoplastic infantile ganglioglioma, desmoplastic infantile astrocytoma, and dysembryoplastic neuroepithelial tumor. (Adapted from WHO) [1]

Neuronal and mixed neuronal-glial tumorss most frequently harbor alterations in BRAF, FGFR1, ZFHX3, SYNE1, and PDGFRA [2].

Most Commonly Altered Genes in Neuronal and Mixed Neuronal-Glial Tumors

BRAF Mutation, BRAF Exon 15 Mutation, BRAF V600E, BRAF Codon 600 Missense, and FGFR1 Mutation are the most common alterations in neuronal and mixed neuronal-glial tumors [2].

Top Alterations in Neuronal and Mixed Neuronal-Glial Tumors

Significant Genes in Neuronal and Mixed Neuronal-Glial Tumors

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
Neuronal and Glio-Neuronal Neoplasm, Neuronal and Mixed Neuronal-Glial Tumor, Neuronal and Glio-Neuronal Tumor
Parent(s)
Neuroepithelial Neoplasm
Children
Extraventricular Neurocytoma, Dysembryoplastic Neuroepithelial Tumor, Rosette-Forming Glioneuronal Tumor, Central Neurocytoma, Gangliocytoma, Dysplastic Cerebellar Gangliocytoma, Papillary Glioneuronal Tumor, Anaplastic Ganglioglioma, Desmoplastic Infantile Ganglioglioma, Cerebellar Liponeurocytoma, Desmoplastic Infantile Astrocytoma, and Ganglioglioma
OncoTree Name
Miscellaneous Neuroepithelial Tumor
OncoTree Code
MNET

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.