# Ependymoma molecular groups (PF-A, PF-B, ZFTA, YAP1, MYCN)

Source: https://onco.cc/terms/ependymoma-molecular-groups/  
OnCo record `ependymoma-molecular-groups` (Term). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Fields

- Kind: Term
- Last checked: 2026-09-17
- Also known as: 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

## Connected records

- technologies: [Methylation classifier for brain tumours](https://onco.cc/technologies/cns-tumour-methylation-classifier/)
- terms: [CDKN2A/B homozygous deletion](https://onco.cc/terms/cdkn2a-homozygous-deletion/), [Extent of resection (RANO resect classes)](https://onco.cc/terms/extent-of-resection/), [FISH / ISH (in situ hybridisation)](https://onco.cc/terms/fish/), [H3 K27M (diffuse midline glioma)](https://onco.cc/terms/h3k27m/), [MYCN amplification](https://onco.cc/terms/mycn-amplification/), [Radiotherapy](https://onco.cc/terms/radiotherapy/)
- cancers: [Ependymoma](https://onco.cc/cancers/ependymoma/), [Spinal cord tumours (intramedullary and intradural)](https://onco.cc/cancers/spinal-cord-tumours/)

---
JSON: https://onco.cc/api/v1/entities/ependymoma-molecular-groups.json