Your Visual Guide Ependymoma

Understand this diagnosis through data, infographics, and evidence based on modern classification and current research.

~1,000
New Cases per Year in the U.S.
Understanding Ependymoma
What Is Ependymoma & How Is It Classified?
Ependymoma arises from ependymal cells lining the ventricles and central canal. WHO 2021 classification emphasizes molecular subtypes defined by ZFTA-RELA fusion, YAP1 fusion, and H3K27me3 loss.
Ependymal Tumor
Anatomic Location?
Supratentorial
Supratentorial (~30%)
ZFTA/YAP1 fusion-defined
ZFTA-RELA fused YAP1 fused
Posterior Fossa
Posterior Fossa (~50%)
PFA (poor) vs PFB (better)
PFA (H3K27me3 loss) PFB (better prognosis)
Spinal
Spinal (~20%)
Best prognosis, myxopapillary
Myxopapillary Classic spinal
Tumor Grading
WHO 2021 Molecular Classification
Ependymomas are classified by anatomic location and molecular subtype, each with unique biology and clinical behavior.
1
Supratentorial ZFTA-RELA fused
Most common supratentorial subtype in children. ZFTA::RELA fusion activates NF-kB pathway. Often aggressive with high recurrence rate.
2
Supratentorial YAP1 fused
Rare supratentorial subtype with YAP1::MAMLD1 fusion. Generally favorable prognosis, especially in young children.
3
Posterior Fossa A (PFA)
Most common in young children (<5 years). H3K27me3 loss, EZHIP overexpression. Worst prognosis among ependymomas.
4
Posterior Fossa B (PFB)
Typically in adolescents/adults. Better prognosis than PFA. Often lateral location in cerebellum.
5
Spinal Ependymoma
Most common intramedullary spinal tumor in adults. WHO Grade 2, often well-circumscribed. Good surgical outcomes.
6
Myxopapillary
Arises in filum terminale/conus medullaris. Slow-growing, WHO Grade 2. Good prognosis after complete resection.
7
Subependymoma
Benign (WHO Grade 1), typically incidental finding. Found in ventricles, most commonly 4th ventricle in adults.
Molecular Markers
The Key Biomarkers
Modern classification depends on specific molecular markers. Each reveals something different about the tumor.
Diagnostic
ZFTA-RELA
ZFTA::RELA Fusion
Defines supratentorial ZFTA subtype. Detectable by FISH or fusion panel. Activates NF-kB signaling.
ST-ZFTA defining
Diagnostic
YAP1
YAP1 Fusion
Defines supratentorial YAP1 subtype. YAP1::MAMLD1 or YAP1::FAM118B fusions. Better prognosis.
ST-YAP1 defining
Prognostic
H3K27me3
H3K27 Trimethylation
Global loss by IHC is hallmark of PFA ependymoma. Mediated by EZHIP overexpression. Poor prognosis.
PFA hallmark
Diagnostic
EZHIP
EZH Inhibitory Protein
Overexpression in PFA causes H3K27me3 loss. Key diagnostic marker distinguishing PFA from PFB.
PFA marker
Prognostic
TERT
TERT Promoter
Promoter mutations in spinal ependymoma. Associated with DNA methylation subgroups and risk stratification.
Spinal subtype
Prognostic
CDKN2A
Cyclin-Dependent Kinase Inhibitor
Homozygous deletion associated with worse outcomes in supratentorial ependymoma.
Poor prognosis
Signs & Symptoms
Ependymoma Signs & Symptoms
Symptoms depend on tumor location (supratentorial, posterior fossa, or spinal) and patient age.

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).

Diagnosis
The Diagnostic Journey
From first scan to molecular subtyping — the path to an ependymoma diagnosis.
1

MRI Brain & Spine

MRI reveals intraventricular or intra-axial mass; spine MRI for staging

2

CT Scan

Shows calcification, hemorrhage; useful in emergency for hydrocephalus

3

Surgical Resection

Maximal safe resection (GTR) is the single most important prognostic factor

4

Neuropathology

WHO grading, histologic pattern (classic, tanycytic, clear cell, papillary)

5

Molecular Testing

FISH for ZFTA-RELA fusion, IHC for H3K27me3 loss, DNA methylation profiling

6

Treatment Plan

Risk-adapted plan based on location, molecular subtype, extent of resection, and age

Treatment
Treatment Options for Ependymoma
Gross total resection is the cornerstone. Radiation plays a key adjuvant role. Chemotherapy has limited efficacy.

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.

Find matching trials →
Care Team
Your Multidisciplinary Care Team
Ependymoma treatment requires a multidisciplinary team with expertise in both pediatric and adult neuro-oncology.
Core Specialists

Neurosurgeon

Primary specialist for surgical resection and skull base approaches

Radiation Oncologist

Plans and delivers radiation therapy and stereotactic radiosurgery

Neuro-oncologist

For higher-grade or refractory ependymomas requiring systemic therapy

Support Team

Neuroradiologist

Specialized imaging interpretation and monitoring

Neuropathologist

Tissue analysis, WHO grading, and molecular profiling

Nurse Navigator

Guides you through appointments, insurance, and logistics

Resources
Support Organizations
Organizations providing information, community, and support for ependymoma patients and families.

CERN Foundation

The Collaborative Ependymoma Research Network — dedicated to ependymoma research and clinical trials.

Visit CERN Foundation →

National Brain Tumor Society

Leading nonprofit investing in research, advocacy, and patient services for all brain tumors.

Visit National Brain Tumor Society →

American Brain Tumor Association

Comprehensive brain tumor information, patient support programs, and research funding.

Visit American Brain Tumor Association →

Pediatric Brain Tumor Foundation

Resources and support for families affected by childhood brain tumors including ependymoma.

Visit Pediatric Brain Tumor Foundation →

Want 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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