Alternate Names: Childhood Ependymoma, Ependymal tumors, Neuroectodermal tumors, primitive.
Ependymoblastoma is a highly malignant brain tumor of childhood and is usually seen in the very young child or infant.
It is rare among brain tumors in general, but these brain tumors are the second most common malignancy in the childhood age group, second only to leukemia.
Ependymoblastoma is part of a group of tumors classified under the central nervous system (CNS) embryonal tumors group.
The symptoms include loss of balance, abnormal speech, general weakness or weakness on one side of the face and double vision.
Infratentorial ependymoblastomas (lower back brain) present with signs and symptoms of increased intracranial pressure and cerebellar signs (coordination symptoms). Supratentorial ependymoblastomas (upper brain) are more likely to present with focal headaches and focal motor signs.
The history and physical examination will suggest the diagnosis of a brain tumor and may suggest the area of the brain involved. The examination will reveal a sicker child in the morning hours who then improves as the day progresses. Although a CT of the head may demonstrate calcification that may not be apparent on a MRI should include the entire spine as well as the brain to identify any spread of the tumor that may already be present. Diagnosis rests on positive tests of the tumor.
Because of the high morbidity associated with whole brain or neuraxis radiation in young children, the therapy for ependymoblastomas is now divided into that for children older than 3 years or 3 years and younger.
Children older than 3 years:
Standard treatment of childhood ependymoblastoma is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes chemotherapy is given at the same time as radiation therapy or after radiation therapy.
Children 3 years or younger:
Standard treatment is usually surgery followed by chemotherapy. Other treatments may include surgery followed by high-dose chemotherapy with bone marrow or stem cell transplant and surgery followed by chemotherapy and low-dose of localized radiation therapy.
Treatment of childhood ependymoblastoma in children 3 years old or younger is often within a clinical trial.
Prognosis is poor, with a 5 year survival rates ranging from 0% to 30%.