Ependymomal Tumor (EPMT)

Overview

Ependymomal tumor (EPMT) is the OncoTree parent node encompassing all ependymoma subtypes and related glial tumors of ependymal lineage, including classic ependymoma (EPM), subependymoma, myxopapillary ependymoma, and ependymomal tumors NOS. This grouping captures the full histologic and molecular spectrum of tumors arising from or differentiating toward ependymal cells.

Cohorts in the corpus

  • mixed_pipseq_2017 — PIPseq pediatric pan-cancer cohort (Columbia University Medical Center), which includes ependymomal tumor cases among 101 high-risk pediatric patients PMID:28007021.

Recurrent alterations

  • PIPseq cohort: EPMT cases included ependymoma (EPM) with C11orf95-RELA fusion (diagnostic and poor-prognosis marker); see EPM page for molecular details PMID:28007021.

Subtypes

  • Ependymoma (EPM) — main subtype with molecular stratification by ZFTA fusion, YAP1 fusion, PFA/PFB methylation class.
  • Subependymoma — slow-growing, typically spinal; NF2 association.
  • Myxopapillary ependymoma — spinal; favorable prognosis.

Therapeutic landscape

  • Surgery and radiotherapy are mainstays; no standard targeted therapy across EPMT as a whole.

Sources

  • PMID:28007021 — Oberg et al. PIPseq pediatric pan-cancer sequencing program (n=101).

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