Meningioma Guia — Seu Guia Visual

Entendendo Meningioma

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

Graus Tumorais

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.

Marcadores Moleculares

Os Principais Biomarcadores

A classificação moderna depende de marcadores moleculares específicos. Cada um revela algo diferente sobre o 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

Sinais & Sintomas

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).

Diagnóstico

A Jornada Diagnóstica

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

Tratamento

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 →

Equipe Médica

Sua Equipe Multidisciplinar

Ependymoma treatment requires a multidisciplinary team with expertise in both pediatric and adult neuro-oncology.

Especialistas Principais

Neurocirurgião

Realiza cirurgia cerebral para remoção do tumor ou biópsia

Radio-oncologista

Planeja e administra radioterapia de precisão

Neuro-oncologista

Especialista em tumores cerebrais que lidera o planejamento do tratamento

Equipe de Apoio

Neurorradiologista

Interpreta exames de imagem cerebral e orienta o diagnóstico por imagem

Neuropatologista

Analisa o tecido tumoral para determinar o tipo e grau exatos

Enfermeiro(a) Navegador(a)

Orienta sobre consultas, convênios e logística

Recursos

Organizações de Apoio

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 →

Seu Guia Visual Meningioma

Posterior Fossa

Supratentorial

Spinal

Cranial Nerves

Raised ICP

Filum Terminale

MRI Brain & Spine

CT Scan

Surgical Resection

Neuropathology

Molecular Testing

Treatment Plan

Surgery

Radiation Therapy

Re-irradiation

Chemotherapy

Observation

Clinical Trials

Neurocirurgião

Radio-oncologista

Neuro-oncologista

Neurorradiologista

Neuropatologista

Enfermeiro(a) Navegador(a)

CERN Foundation

National Brain Tumor Society

American Brain Tumor Association

Pediatric Brain Tumor Foundation

Quer Explorar Estudos Clínicos?