Not Found
Locations

Find information on medical topics, symptoms, drugs, procedures, news and more, written for the health care professional.

* This is the Professional Version. *

Ependymomas

By John J. Gregory, Jr., MD, Physician;Physician, Goryeb Children's Hospital, Atlantic Health;UMDNJ New Jersey Medical School

Click here for
Patient Education

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.

Symptoms and Signs

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

  • MRI

  • 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

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

  • Surgical resection, usually followed by radiation therapy

  • Sometimes chemotherapy

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.

* This is the Professional Version. *