Overview

NCI Definition: An aggressive malignant embryonal neoplasm arising from the central nervous system. It is characterized by the presence of multilayered rosettes formation and the presence of amplification of the C19MC region on chromosome 19 (19q13.42). [1]

Significant Genes in Embryonal Tumor with Multilayered Rosettes, C19MC-Altered

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
ETMR, C19MC-Altered, Embryonal Tumor with Abundant Neuropil and True Rosettes, Ependymoblastoma, Embryonal Tumor with Multilayered Rosettes iwth C19MC Amplification, Embryonal Tumor with Multilayered Rosettes
Parent(s)
Central Nervous System Embryonal Neoplasm
Children
Adult Ependymoblastoma, Childhood Cerebral Ependymoblastoma, Childhood Supratentorial Ependymoblastoma, and Childhood Infratentorial Ependymoblastoma
OncoTree Name
Embryonal Tumor with Abundant Neuropil and True Rosettes
OncoTree Code
ETANTR

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 6. 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.