Overview

NCI Definition: A malignant neoplasm of embryonal origin, arising from the central nervous system. It usually affects children. Representative examples include ependymoblastoma, medulloblastoma, and atypical teratoid/rhabdoid tumor. [1]

Significant Genes in Central Nervous System Embryonal Neoplasm

APC +

CDK6 +

CTDNEP1 +

CTNNB1 +

DDX3X +

GLI2 +

H3F3A +

IDH1 +

IDH2 +

KDM6A +

KMT2C +

KMT2D +

LRP1B +

MDM4 +

MYC +

MYCL +

MYCN +

OTX2 +

PPM1D +

PTCH1 +

PTEN +

PVT1 +

RELA +

SHH +

SMARCA4 +

SMO +

SNCAIP +

SUFU +

TERT +

TP53 +

YAP1 +

ZMYM3 +

Disease Details

Synonyms
Embryonal Tumor of the Central Nervous System, CNS Embryonal Tumor
Parent(s)
Malignant Central Nervous System Neoplasm
Children
Central Nervous System Neuroblastoma, Pineoblastoma, Spinal Cord Embryonal Tumor, Not Otherwise Specified, Embryonal Tumor with Multilayered Rosettes, C19MC-Altered, Medulloblastoma, Central Nervous System Primitive Neuroectodermal Neoplasm, Atypical Teratoid/Rhabdoid Tumor, Medulloepithelioma, Central Nervous System Ganglioneuroblastoma, and Embryonal Tumor with Multilayered Rosettes, Not Otherwise Specified
OncoTree Name
Embryonal Tumor
OncoTree Code
EMBT

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.