Overview

NCI Definition: A group of neoplasms which arise from the ependymal lining of the cerebral ventricles and from the remnants of the central canal of the spinal cord. Ependymal tumors occur predominantly in children and young adults with varied morphological features and biological behavior. There are 4 types: ependymoma, anaplastic ependymoma, myxopapillary ependymoma and subependymoma. (WHO) [1]

Ependymal tumors most frequently harbor alterations in RELA, C11orf95, CDKN2A, KMT2D, and ARID1B [2].

Most Commonly Altered Genes in Ependymal Tumor

C11orf95-RELA Fusion, RELA Fusion, KMT2D Mutation, CDKN2B Loss, and CDKN2A Loss are the most common alterations in ependymal tumor [2].

Top Alterations in Ependymal Tumor

Significant Genes in Ependymal Tumor

H3F3A +

Disease Details

Synonyms
Ependymal Neoplasm
Parent(s)
Glioma
Children
Subependymoma, Ependymal Tumor of Brain, Anaplastic Ependymoma, Myxopapillary Ependymoma, Ependymoma, RELA Fusion-Positive, Brain Stem Ependymoma, and Ependymoma
OncoTree Name
Ependymomal Tumor
OncoTree Code
EPMT

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.