ALTERNATE NAMES

Anaplastic Ependymal Cancer; Anaplastic Ependymal Carcinoma; Anaplastic Ependymoma Cancer; Anaplastic Ependymoma Carcinoma; Anaplastic Tumor; Anaplastic Ependymal Cancer; Anaplastic Ependymal Tumor; Malignant Ependymoma

DESCRIPTION

Anaplastic Ependymoma is a malignant tumor that forms in the central nervous system (including the brain and spinal cord). An ependymoma is anaplastic if the cells grow very quickly and are significantly unusual in shape.

The symptoms of an anaplastic ependymoma depend on the age of the person and the size and location of the tumor. Anaplastic ependymoma is very aggressive and is classed by the World Health Organization (WHO) as a Grade III central nervous system cancer (CNS).

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

Diagnostic testing: The diagnosis of anaplastic ependymoma is made by:

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  Magnetic resonance imaging (MRI) or computed tomography (CT) scan;

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  Tissue biopsy; and

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  Tumor resection (removal).

Physical findings: Symptoms of anaplastic ependymoma may include:

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  Headaches;

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  Nausea;

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  Vision changes;

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  Seizures; and

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  Changes in mood or personality.

ICD-9: 191.9

ICD-10: C71.9

PROGRESSION

Ependymomas can occur at any age. When ependymomas occur in children, they are frequently located in the brain (intracranial). In adults, ependymomas are often found in the spinal cord.

The exact cause of anaplastic ependymoma is not known, but it is thought that certain changes (acquired pathogenic variants, also known as somatic genetic changes) in specific genes in some cells of the body allow the cells to begin growing quickly.

For Grade III anaplastic ependymomas the prognosis is poor. The relative 5-year survival rate for ependymoma is about 60% but many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment.

TREATMENT

The treatment of anaplastic ependymoma is surgical if the tumor is localized and technically resectable. If surgery is not an option, chemotherapy and radiation therapy are alternative treatments.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Imaging reports such as CT scan or MRI scan; and

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  Biopsy or needle aspiration information found in operative notes, pathology reports, summaries of hospitalization or other medical reports that include details of the surgical and pathological findings.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.13 A 2

113.13 B

Anaplastic ependymoma is a Grade III CNS cancer. Response to treatment is not necessary to meet the listing.

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