Ependymomas are the 3rd most common central nervous system tumor in children Overview of Brain Tumors in Children Brain tumors are the most common solid cancer in children 15 years of age and are the 2nd leading cause of childhood death due to cancer. Diagnosis is typically by imaging (usually MRI) and... read more (after astrocytomas Astrocytomas Astrocytomas are childhood central nervous system tumors that develop from astrocytes. Diagnosis is based on MRI. Treatment is a combination of surgical resection, radiation therapy, and chemotherapy... read more and medulloblastomas Medulloblastoma Medulloblastomas are invasive and rapidly growing childhood central nervous system tumors that develop in the posterior fossa (containing the brain stem and cerebellum). Diagnosis is based on... read more ), representing 10% of pediatric brain tumors. Mean age at diagnosis is 6 years; however, about 30% of ependymomas occur in children < age 3 years.
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.
Symptoms and Signs of Ependymomas
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.
Diagnosis of Ependymomas
Histologic evaluation of biopsy specimen or entire resected tumor
Diagnosis of ependymoma is based on MRI.
Definitive diagnosis is made using tumor tissue obtained by biopsy or ideally by gross total resection of the tumor at initial presentation.
Prognosis for Ependymomas
Survival rate depends on age and on how much of the tumor can be removed:
Total or near-total removal: 51 to 80% survival
Less than 90% removal: 0 to 26% survival
Children who survive are at risk of neurologic deficits.
Treatment of Ependymomas
Surgical resection, usually followed by radiation therapy
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 patients with ependymomas who do not receive radiation 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.