ALTERNATE NAMES

Anaplastic Meningioma; Grade III Meningioma; Grade 3 Meningioma; Malignant Meningioma; Malignant Meningioma with Rhabdoid Features; Meningioma Grade 3; Meningioma Grade III; Meningeal Tumor; Papillary Meningioma; Rhabdoid Meningioma; Rhabdoid Morphology Meningioma; WHO Grade 3 Meningioma

DESCRIPTION

Meningioma is a type of tumor that originates from the membranes that cover and protect the brain and spinal cord (meninges). Rhabdoid meningioma, anaplastic meningioma, and papillary meningioma are rare and aggressive subtypes of meningiomas characterized by rapid growth, high incidence of recurrence, and poor survival compared to other meningiomas. They are primary central nervous system (CNS) tumors that are classified as Grade III by the World Health Organization (WHO). A WHO Grade III classification means the tumor is malignant, fast growing, and often spreads to other parts of the body. Excluding brain metastases from other organs, it is the most common intracranial tumor and the most common primary brain tumor.

Only 1 to 4 percent of all meningiomas are grade III. The cause is not known. Meningiomas can occur at any age, but they are most common in adults aged 65 or older, and the risk increases with age. Men are more likely to be diagnosed than women. Although extremely rare, these tumors can occur in children. The survival is shorter in children compared to adults.

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

Diagnostic testing: Diagnostic testing for WHO Grade III meningiomas may include:

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  Physical examination;

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  Medical and family history;

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  Neurological examination;

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  Brain magnetic resonance imaging (MRI);

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  Computed tomography (CT) scan.

Physical findings: Symptoms for WHO Grade III meningiomas include:

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  Behavioral or personality changes;

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  Changes in your vision, such as double vision, blurriness, or loss of vision;

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  Dizziness;

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  Headaches;

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  Hearing loss;

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  Loss of smell;

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  Memory problems;

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  Muscle weakness in certain areas of your body;

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  Nausea and vomiting;

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  Overactive or overresponsive reflexes (hyperreflexia);

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  Paralysis in certain area of your body; and

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  Seizures.

ICD-9: 192.1

ICD-10: C70.9

PROGRESSION

Prognosis for WHO Grade III meningiomas is poor due to rapid growth and high frequency of recurrence.

TREATMENT

The primary treatment for WHO Grade III meningiomas is surgical resection, which is the removal of a tumor. The goal of surgery is to obtain tissue to determine the tumor type and remove as much of it as possible without causing more symptoms. The extent of the resection largely impacts the rates of recurrence. Surgical removal (resection) might be combined with radiotherapy, especially if complete resection of the tumor is not possible. Radiation therapy alone can sometimes be used to treat small tumors.

Adjuvant radiotherapy is also used for cancerous meningiomas. It improves control of the tumor’s growth with longer progression-free survival and overall survival. Adjuvant therapy, sometimes called helper therapy, targets cancer cells that primary treatment didn’t destroy.

Palliative care may be used to help manage physical, emotional, and social symptoms and side effects caused by treatment. Palliative treatments often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies.

Suggested MER for Evaluation:

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

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  Family medical history; and

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  Results of imaging (CT scan and MRI).

Suggested Listings for Evaluation:

Meets

13.13 A 2

113.13 B

Tumor is WHO Grade III and meets listing.

Equals