EPENDYMOBLASTOMA (CHILD BRAIN CANCER)

ALTERNATE NAMES

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

DESCRIPTION

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.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

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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  Computed tomography (CT) or magnetic resonance imaging (MRI) scans of the brain and spine;

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  Lumbar puncture; and

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  Biopsy.

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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  Lack of coordination;

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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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  Double vision;

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  Increased intracranial pressure, seizures; and

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  Headaches.

ICD-9: 191.9

ICD-10: C71.9

TREATMENT

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.

PROGRESSION

Prognosis is poor, with a 5-year survival rates ranging from 0% to 30%.

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.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.13 A 1

113.13 A

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