Program Operations Manual System (POMS)
TN 54 (09-22)
DI 23022.150 Ependymoblastoma (Child Brain Cancer)
COMPASSIONATE ALLOWANCES INFORMATION
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EPENDYMOBLASTOMA (CHILD BRAIN
CANCER)
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ALTERNATE NAMES
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Childhood Ependymoma; Ependymal Tumors; Neuroectodermal Tumors, Primitive; Embryonal
Tumor with Multilayer Rosettes, C19MC-Altered; Embryonal Tumor with Multilayer Rosettes,
Not Otherwise Specified (NOS); ETMR
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: The diagnosis of ependymoblastoma is made by a combination of:
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Clinical history and physical examinations;
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Neurological examination;
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CT/MRI scans of the brain and spine;
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Physical findings: Children with an ependymoblastoma may present with:
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Loss of balance, difficulties with walking, worsening handwriting, or abnormal speech;
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General weakness or weakness on one side of the face;
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Unusual sleepiness or changes in energy level;
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Increased intracranial pressure, seizures; and
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ICD-9: 191.9
ICD-10: C71.9
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TREATMENT
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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.
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PROGRESSION
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Prognosis is poor, with a 5- year survival rates ranging from 0% to 30%.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Pathology reports of the cancer;
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Cytology report of the cerebrospinal fluid (CSF);
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Neuroradiological studies including CT or MRI of the brain; and
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Neuroradiological studies of the entire neuraxis.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.13 A 1
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113.13 A
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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