Overview

NCI Definition: A benign glial-neuronal neoplasm. It is usually supratentorial, located, generally, in the cortex and occurs in children and young adults with a long-standing history of partial seizures. A histologic hallmark of this tumor is the 'specific glioneuronal element', characterized by columns, made up of bundles of axons, oriented perpendicularly to the cortical surface. (Adapted from WHO) [1]

Dysembryoplastic neuroepithelial tumors most frequently harbor alterations in FGFR1, CUX1, BRCA2, ZMYM2, and TRIM24 [2].

Most Commonly Altered Genes in Dysembryoplastic Neuroepithelial Tumor

FGFR1 Mutation, FGFR1 Exon 14 Mutation, ZMYM2-FGFR1 Fusion, TRIM24-FGFR1 Fusion, and TPR-FGFR1 Fusion are the most common alterations in dysembryoplastic neuroepithelial tumor [2].

Top Alterations in Dysembryoplastic Neuroepithelial Tumor

Significant Genes in Dysembryoplastic Neuroepithelial Tumor

MYCN +

Disease Details

Synonyms
Dysembryoplastic Neuroepithelial Neoplasm, DNET, DNT
Parent(s)
Neuronal and Mixed Neuronal-Glial Tumors
OncoTree Name
Dysembryoplastic Neuroepithelial Tumor
OncoTree Code
DNT

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.