Program Operations Manual System (POMS)
TN 13 (09-15)
DI 23022.150 Ependymoblastoma (Child Brain Cancer)
COMPASSIONATE ALLOWANCE INFORMATION
EPENDYMOBLASTOMA (CHILD BRAIN CANCER)
Ependymoblastoma is a highly malignant brain cancer of childhood and is usually seen in the very young child or infant. It is rare among brain cancers in general, but these brain cancers are the second most common malignancy in the childhood age group, second only to leukemia.
Ependymoblastoma is part of a group of cancers classified under the central nervous system (CNS) embryonal cancers 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.
Childhood Ependymoma; Ependymal Tumors; Neuroectodermal Tumors, Primitive.
DIAGNOSTIC TESTING AND CODING
The history and physical examination will suggest the diagnosis of a brain cancer 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 cancer that may already be present. Diagnosis rests on positive tests of the cancer.
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%.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Pathology reports of the cancer.
If available, cytology of the cerebrospinal fluid (CSF).
Additional supporting evidence includes neuroradiological studies including CT or MRI of the brain.
If available, neuroradiological studies of the entire neuraxis.
Suggested Listings for Evaluation:
13.13 A 1
* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.