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Ependymomas are now named by where they arise and their molecular group: posterior fossa group A (young children, hardest to cure, worse still with 1q gain), group B (older patients, mostly cured with surgery and radiotherapy), supratentorial ZFTA-fusion and YAP1-fusion tumours, and a rare aggressive MYCN-amplified spinal type; the group sets the intensity of radiotherapy and the follow-up.
What is measured: the molecular group, which WHO 2021 makes part of the diagnosis. How: DNA methylation profiling (Heidelberg classifier) is the reference; surrogates are H3K27me3 immunohistochemistry loss with EZHIP positivity for PF-A, FISH or RNA sequencing for ZFTA (formerly C11orf95)::RELA and YAP1::MAMLD1 fusions, L1CAM and nuclear p65 staining for ZFTA tumours, MYCN FISH for spinal tumours, and copy-number for 1q gain (adverse in PF-A), 6q loss (a PF-A subtype) and CDKN2A deletion (adverse in ZFTA). Types: supratentorial ZFTA fusion-positive and YAP1 fusion-positive; posterior fossa PFA and PFB; spinal, spinal MYCN-amplified, myxopapillary and subependymoma. What a result changes: for PF-A, gross total resection is the strongest factor (second-look surgery is standard), conformal radiotherapy to 59.4 Gy is given even under age 3 (ACNS0831, SIOP Ependymoma II), 1q gain places a child in high-risk arms, and EZHIP or EZH2-directed therapy is in early trials; PF-B after complete resection is being observed without radiotherapy in trials; YAP1 tumours do well; chemotherapy has a limited role. Where it matters: ependymoma and the spinal cord tumour page.
Shares Methylation classifier for brain tumours, Extent of resection (RANO resect classes), CDKN2A/B homozygous deletion, FISH / ISH (in situ hybridisation).
Shares Methylation classifier for brain tumours, Extent of resection (RANO resect classes), MYCN amplification.
Shares MYCN amplification, FISH / ISH (in situ hybridisation).
Shares MYCN amplification, FISH / ISH (in situ hybridisation).
Shares MYCN amplification, FISH / ISH (in situ hybridisation).
Shares Spinal cord tumours (intramedullary and intradural), Ependymoma.
Shares MYCN amplification, Radiotherapy.
Shares Extent of resection (RANO resect classes), CDKN2A/B homozygous deletion.