Ependymoma - molecular classification, management and prognosis

Subspecialty: Neuro-oncology

Type: Clinical Summary

Ependymoma is a heterogeneous group of glial tumors whose clinical management and prognosis are now driven primarily by integrated molecular classification rather than histology alone; key biological drivers include fusion oncoproteins (eg, ZFTA:RELA, YAP1 fusions), epigenetic signatures (eg, Posterior Fossa Group A [PFA] CpG island methylation), copy-number alterations (eg, 1q gain, 6q loss), and oncogene amplifications (eg, MYCN). Retrospective series and complementary molecular studies review real‑world impacts of routine DNA methylation array profiling, subgroup‑specific biology, diagnosti…

Source Papers

Molecular Classification and Therapeutic Targets in Ependymoma. (2021)
Impact of molecular classification on prognosis in children and adolescents with spinal ependymoma: Results from the HIT-MED database. (2024)
Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors. (2018)
Pediatric ependymoma in the molecular era: real-world experience with diagnostic correlation and long-term clinical outcomes. (2026)