Ependymoblastoma (Child Brain Tumor) Information

Ian C. Langtree - Writer/Editor for Disabled World (DW)
Published: 2009/04/01 - Updated: 2023/01/28
Topic: Brain Cancer and Tumors - Publications List

Page Content: Synopsis - Introduction - Main

Synopsis: Ependymoblastoma is a highly malignant brain tumor of childhood and is usually seen in the very young child or infant. Ependymoblastoma is part of a group of tumors classified under the central nervous system (CNS) embryonal tumors group.

Introduction

Ependymoblastoma Alternate Names: Childhood Ependymoma; Ependymal Tumors; Neuroectodermal Tumors, Primitive; Embryonal Tumor with Multilayer Rosettes, C19MC-Altered; Embryonal Tumor with Multilayer Rosettes, Not Otherwise Specified (NOS); ETMR.

The U.S. Social Security Administration (SSA) has included Ependymoblastoma (Child Brain Tumor) as a Compassionate Allowance to expedite a disability claim.

Main Item

Ependymoblastoma is a highly malignant childhood brain tumor usually seen in a very young child or infant. It is rare among brain tumors in general. Still, 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.

Ependymoblastoma Symptoms

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 improve as the day progresses.

Although a head CT may demonstrate calcification that may not be apparent on an MRI should include the entire spine and 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 three or three years.

Ependymoblastoma Treatment

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 simultaneously 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 localized radiation therapy.

Treatment of childhood ependymoblastoma in children three years or younger is often within a clinical trial.

Ependymoblastoma prognosis is poor, with a five-year survival rate ranging from 0% to 30%.

Author Credentials: Ian was born and grew up in Australia. Since then, he has traveled and lived in numerous locations and currently resides in Montreal, Canada. Ian is the founder, a writer, and editor in chief for Disabled World. Ian believes in the Social Model of Disability, a belief developed by disabled people in the 1970s. The social model changes the focus away from people's impairments and towards removing barriers that disabled people face daily. To learn more about Ian's background, expertise, and achievements, check out his bio.