Understanding Ependymoma,
from diagnosis to care
Ependymoma arises from ependymal cells lining the ventricles and central canal. WHO 2021 classification emphasizes molecular subtypes with distinct biology, location, and prognosis.
New Cases per Year in the U.S.
Diagnosis combines imaging, pathology, and molecular features to guide care.
Most plans combine surgery, radiation, systemic therapy, and ongoing supportive care.
What is ependymoma, and how is it classified?
The subtypes below summarise how this condition is classified in modern neuro-oncology — each behaves differently and is treated differently.
Supratentorial ZFTA-RELA fused
Most common supratentorial subtype in children. ZFTA::RELA fusion activates NF-kB pathway. Often aggressive with high recurrence rate.
Supratentorial YAP1 fused
Rare supratentorial subtype with YAP1::MAMLD1 fusion. Generally favorable prognosis, especially in young children.
Posterior Fossa A (PFA)
Most common in young children (<5 years). H3K27me3 loss, EZHIP overexpression. Worst prognosis among ependymomas.
Posterior Fossa B (PFB)
Typically in adolescents/adults. Better prognosis than PFA. Often lateral location in cerebellum.
Spinal Ependymoma
Most common intramedullary spinal tumor in adults. WHO Grade 2, often well-circumscribed. Good surgical outcomes.
Myxopapillary
Arises in filum terminale/conus medullaris. Slow-growing, WHO Grade 2. Good prognosis after complete resection.
Subependymoma
Benign (WHO Grade 1), typically incidental finding. Found in ventricles, most commonly 4th ventricle in adults.
The biomarkers that define your tumor
Modern classification depends on specific molecular markers — each revealing something different about the tumor and its likely behaviour.
Defines supratentorial ZFTA subtype. Detectable by FISH or fusion panel. Activates NF-kB signaling.
ST-ZFTA definingDefines supratentorial YAP1 subtype. YAP1::MAMLD1 or YAP1::FAM118B fusions. Better prognosis.
ST-YAP1 definingGlobal loss by IHC is hallmark of PFA ependymoma. Mediated by EZHIP overexpression. Poor prognosis.
PFA hallmarkOverexpression in PFA causes H3K27me3 loss. Key diagnostic marker distinguishing PFA from PFB.
PFA markerPromoter mutations in spinal ependymoma. Associated with DNA methylation subgroups and risk stratification.
Spinal subtypeHomozygous deletion associated with worse outcomes in supratentorial ependymoma.
Poor prognosisHow ependymoma can present
Symptoms vary by tumor location and size. This is general information — only your care team can interpret your situation.
Posterior Fossa
Headache, nausea, vomiting from hydrocephalus. Most common presentation in children.
Supratentorial
Seizures, headache, focal neurological deficits depending on tumor location.
Spinal
Back pain, progressive myelopathy, sensory changes, bowel/bladder dysfunction.
Cranial Nerves
Lower cranial nerve palsies from 4th ventricle floor invasion (PF ependymomas).
Raised ICP
Papilledema, 6th nerve palsy, altered consciousness, macrocephaly in infants.
Filum Terminale
Low back pain, leg weakness, saddle anesthesia (myxopapillary ependymoma).
Treatment options for Ependymoma
Treatment depends on tumor type, grade, location, and overall health. Most plans combine several approaches.
Surgery
Gross total resection (GTR) is the strongest prognostic factor. Second-look surgery for residual disease may be beneficial.
Radiation Therapy
Focal conformal radiation after resection for children >1 year. Proton therapy preferred to minimize late effects.
Re-irradiation
For recurrent ependymoma, re-irradiation can provide durable disease control with acceptable toxicity.
Chemotherapy
Limited efficacy in ependymoma. Used in infants to delay radiation. Temozolomide, platinum-based regimens studied.
Observation
For completely resected myxopapillary and subependymoma (WHO Grade 1-2) in select cases.
Clinical Trials
Molecular subtype-directed trials, targeted therapies, and immunotherapy approaches under investigation.
Your multidisciplinary care team
Care is delivered by a team — specialists who diagnose and treat, and those who protect day-to-day quality of life.
Neurosurgeon
Performs brain or spine surgery for tumor removal or biopsy.
Best for: resection strategy, biopsy decisions, and surgery-related risk.
Neuro-oncologist
Brain-tumor specialist who leads treatment planning.
Best for: integrating pathology, imaging, medication, and trial options into one plan.
Radiation oncologist
Plans and delivers precision radiation therapy.
Best for: dose planning, side effects, and timing around surgery or systemic therapy.
Nurse navigator
Guides you through appointments, insurance, and logistics.
Best for: referrals, scheduling, records, and getting the right people in the room.
Oncology nurse
Day-to-day care coordination and symptom management.
Best for: new symptoms, medication questions, and urgent care coordination.
Mental health
Psychological support for patients and caregivers.
Best for: coping with uncertainty, caregiver strain, and adjustment after diagnosis.
You are not alone
These organisations provide information, community, and support for brain & spine tumor patients and caregivers.
CERN Foundation
The Collaborative Ependymoma Research Network — dedicated to ependymoma research and clinical trials.
Visit site ResourceNational Brain Tumor Society
Leading nonprofit investing in research, advocacy, and patient services for all brain tumors.
Visit site ResourceAmerican Brain Tumor Association
Comprehensive brain tumor information, patient support programs, and research funding.
Visit site ResourcePediatric Brain Tumor Foundation
Resources and support for families affected by childhood brain tumors including ependymoma.
Visit site
Ready to explore clinical trials?
Search clinical trials for ependymoma and find the ones that match your specific diagnosis and molecular subtype.
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