A CNS tumor that occurs in the CNS in children and adults.
The incidence is approximately 0.2 to 0.4 per 100,000 individuals.
Arise most commonly within the supratentorial brain (cerebral hemispheres) or posterior fossa (cerebellum and brainstem), and spine.
Ependymomas is the third most common brain tumors of childhood, after gliomas and medulloblastomas, and account for 5 to 10% of CNS tumors in children.
90% of ependymomas are intracranial with most arising in the posterior fossa, and the remainder in the spine.
There are at least nine molecular subtypes classified on histologic characteristics, molecular features and location.
Ependymomas are classified as grade 1, 2 or 3 according to the degree of anaplasia.
Spinal ependymomas occur most commonly in adults.
Treatment is maximum safe resection followed by radiation therapy.
Survival rates approximately 80%.
Composed of at least 9 defferent subtypes by molecular analysis.
Account for 8-10% of brain tumors in children and 1-3% of adult brain tumors.
60% of ependymomas occur in children less than 16 years of age.
Approximately half of cases in childhood are under the age of 3 years.
Frequent gene fusions define supratentorial ependymoma namely, C11ORF95-RELA.
Supratentorial ependymomas are characterized by two oncologic molecular fusions – the C 11orf95-RELA fusion occurs and 70% of cases causing constitutive activation of nuclear factor kappa beta signaling pathway, and the YAP1 fusion.
Mainstay of treatment is resection and adjuvant radiotherapy.
5 year progression free survival ranges between 30-60% , with worse prognosis for patients with residual disease after surgery, anaplastic changes, or age younger than 3 years.
Nearly 50% of patients have recurrent disease after primary treatment.
5-year survival in children 14-60% and in adults greater than 55-90%
Spinal cord ependymomas have a more favorable prognosis than intracranial ependymal tumors.
Spinal cord ependymomas derived from neuroectoderm are very rare, 2-4per million.
The fourth ventricle is typical location, but may be intra or extra-medullary in location
Spinal cord ependymomas peak incidence at ages 5 and 35 years.
Increased incidence with type 2 neurofibromatosis.
Platinum compounds are considered to be the best chemotherapeutic agents for recurrent disease, but they do not increase survival.