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Ependymoma

Glial tumor arising from ependymal cells of the brain ventricles and spinal cord.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Onkoloji department. Book Appointment →

What is Ependymoma?

Ependymoma is a glial tumor arising from ependymal cells lining the cerebral ventricles, central canal of the spinal cord, and filum terminale. It accounts for around 6-9% of all CNS tumors in children (third most common pediatric brain tumor) and 2-3% in adults. Anatomic locations are intracranial (supratentorial, posterior fossa) or spinal (intramedullary, filum terminale, myxopapillary).

WHO 2021 classification integrates molecular features. Supratentorial ependymomas are subdivided into ZFTA fusion-positive (formerly RELA-fusion, with worse prognosis) and YAP1 fusion-positive. Posterior fossa ependymomas split into Group A (PFA, infants, poor prognosis, EZHIP overexpression) and Group B (PFB, older children/adults, better prognosis). Spinal ependymomas include MYCN-amplified (aggressive) and the unique myxopapillary (filum terminale).

Patients present with hydrocephalus from CSF obstruction (headache, vomiting, papilledema in posterior fossa), focal deficits, ataxia, or back pain (spinal). Diagnosis combines MRI brain and entire spine, CSF cytology for staging, and surgical biopsy with integrated molecular workup. Maximal safe gross-total resection is the strongest prognostic factor. Adjuvant focal radiotherapy is standard for non-myxopapillary; whole-CSF radiotherapy is rarely used. Chemotherapy benefit is limited but used in selected pediatric protocols. Molecular-targeted trials are ongoing.

Symptoms

Headache (often morning, worsening)
Nausea, vomiting (raised intracranial pressure)
Papilledema
Vision changes, diplopia
Ataxia, balance problems
Cranial nerve palsies (lower CN in posterior fossa)
Macrocephaly in infants
Failure to thrive
Seizures (supratentorial)
Hemiparesis or focal neurologic deficit
Back pain (spinal)
Lower extremity weakness or numbness
Bowel and bladder dysfunction (cauda equina with myxopapillary)
Torticollis (posterior fossa)
Behavioral or cognitive changes
Drop metastases (CSF spread, especially myxopapillary)

Risk Factors

Younger age (most pediatric)
NF2 (spinal ependymomas common)
Turcot syndrome (rare)
Constitutional mismatch repair deficiency
Prior cranial radiation
Familial cases (rare)
ZFTA fusion (somatic, not inherited)
Male sex (slight predominance)
RELA fusion supratentorial (worse prognosis)
Posterior fossa Group A (infants, EZHIP+)
MYCN amplification (spinal aggressive)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent headache, especially morning vomiting
  • Macrocephaly in infants
  • Loss of milestones
  • New seizures
  • Persistent ataxia
  • Cranial nerve palsy
  • Papilledema
  • Severe back pain in child or young adult
  • Bowel/bladder dysfunction with leg symptoms
  • Family history of NF2
  • Failure to thrive in infant

Treatment Methods

01
Pediatric or adult neuro-oncology referral
02
MRI brain and entire spine with contrast
03
CSF cytology after sufficient post-op interval
04
Maximal safe gross-total resection (strongest prognostic factor)
05
Tissue molecular workup: ZFTA/RELA fusion, YAP1 fusion, EZHIP, methylation profiling
06
Integrated WHO 2021 molecular diagnosis
07
Adjuvant focal proton or photon radiotherapy 54-59.4 Gy (most subgroups, even after gross-total resection)
08
Avoid radiotherapy in infants under 12 months when possible (consider chemotherapy bridging)
09
Craniospinal irradiation reserved for disseminated disease
10
Pediatric chemotherapy protocols (vincristine-cisplatin-cyclophosphamide-etoposide) when delaying RT in young children
11
Second-look surgery if residual after initial resection
12
Myxopapillary spinal: gross-total resection if feasible; adjuvant RT for incomplete resection
13
Recurrent disease: re-resection, re-irradiation, salvage chemotherapy, clinical trial enrollment
14
Targeted therapy trials (FAK, EZH2 inhibitors) for selected molecular subgroups
15
VP shunt or ETV for hydrocephalus
16
Steroid management for cerebral edema
17
Anticonvulsants for seizures
18
Long-term endocrine, growth, neurocognitive, hearing surveillance
19
Speech, occupational, physical therapy
20
Posterior fossa syndrome management (mutism, ataxia, behavior)
21
Psychosocial and educational support
22
Genetic counseling for NF2 or syndromic features
23
Long-term follow-up MRI (ependymomas have late recurrences)
24
Survivorship clinic for late effects

Which Department to Visit?

You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.