Ependymomas are slow-growing CNS tumors that involve the ventricular system. Diagnosis is based on MRI and biopsy. Treatment is a combination of surgery, radiation therapy, and chemotherapy.
Ependymomas are the 3rd most common CNS tumor in children (after astrocytomas and medulloblastomas), representing 10% of pediatric brain tumors. Mean age at diagnosis is 6 yr; however, about 30% of ependymomas occur in children < 3 yr.
Ependymomas are derived from the ependymal lining of the ventricular system. Up to 70% of ependymomas occur in the posterior fossa; both high-grade and low-grade tumors in the posterior fossae tend to spread locally to the brain stem.
Initial symptoms are typically related to increased intracranial pressure. Infants may present with developmental delay and irritability.
Changes in mood, personality, or concentration may occur. Seizures, balance and gait disturbances, or symptoms of spinal cord compression (eg, back pain, loss of bladder and bowel control) may occur.
Surgical resection is critical, and the degree of resection is one of the most important prognostic factors.
Radiation therapy has been shown to increase survival and should be given after surgery; however, a small subset of ependymomas can potentially can be cured by surgery alone.
Chemotherapy has not been clearly shown to improve survival but, in some children, may be used to shrink the tumor before gross total resection or a second-look surgery.