{"entity":{"id":"ependymoma-molecular-groups","kind":"term","name":"Ependymoma molecular groups (PF-A, PF-B, ZFTA, YAP1, MYCN)","aka":["PF-A","PFA ependymoma","posterior fossa group A","PF-B","PFB ependymoma","posterior fossa group B","ZFTA fusion","ZFTA::RELA","RELA fusion","C11orf95-RELA","YAP1 fusion","YAP1::MAMLD1","supratentorial ependymoma ZFTA fusion-positive","MYCN-amplified spinal ependymoma","EZHIP","H3K27me3 loss in ependymoma","1q gain ependymoma","chromosome 1q gain","6q loss"],"tldr":"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.","summary":"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.","asOf":"2026-09-17","links":[],"tags":[],"related":["cns-tumour-methylation-classifier","h3k27m","mycn-amplification","extent-of-resection","fish","radiotherapy","cdkn2a-homozygous-deletion"],"cancers":["ependymoma","spinal-cord-tumours"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"category":"Biomarkers"},"route":"/terms/ependymoma-molecular-groups/","neighbours":{"technology":[{"id":"cns-tumour-methylation-classifier","kind":"technology","name":"Methylation classifier for brain tumours","route":"/technologies/cns-tumour-methylation-classifier/"}],"term":[{"id":"cdkn2a-homozygous-deletion","kind":"term","name":"CDKN2A/B homozygous deletion","route":"/terms/cdkn2a-homozygous-deletion/"},{"id":"extent-of-resection","kind":"term","name":"Extent of resection (RANO resect classes)","route":"/terms/extent-of-resection/"},{"id":"fish","kind":"term","name":"FISH / ISH (in situ hybridisation)","route":"/terms/fish/"},{"id":"h3k27m","kind":"term","name":"H3 K27M (diffuse midline glioma)","route":"/terms/h3k27m/"},{"id":"mycn-amplification","kind":"term","name":"MYCN amplification","route":"/terms/mycn-amplification/"},{"id":"radiotherapy","kind":"term","name":"Radiotherapy","route":"/terms/radiotherapy/"}],"cancer":[{"id":"ependymoma","kind":"cancer","name":"Ependymoma","route":"/cancers/ependymoma/"},{"id":"spinal-cord-tumours","kind":"cancer","name":"Spinal cord tumours (intramedullary and intradural)","route":"/cancers/spinal-cord-tumours/"}]}}