CPC

Choroid Plexus Carcinoma is a rare malignant cancer of the brain. It grows aggressively, invading surrounding tissue and impairing normal brain function.

The condition is so named because it typically occurs in the ventricles of the choroid plexus structure, which produces and secretes most of the body’s cerebrospinal fluid. It is also occasionally observed originating in the spinal cord.

Choroid Plexus Carcinoma can affect people at any age, but it is usually seen in infants within the first year of life. It is a Grade III tumor, not to be confused with Choroid Plexus Papilloma (Grades I and II), which is non-cancerous.

Diagnostic testing: Diagnosis of Choroid Plexus Carcinoma is made based on:

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  Physical and neurological examination;

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  Brain imaging including magnetic resonance imaging (MRI) and computerized tomography (CT) scans; and

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  Genetic testing.

Choroid Plexus Carcinoma tumors appear as a distinct “cauliflower-like” shape on an MRI.

Physical findings: Common physical symptoms of Choroid Plexus Carcinoma include:

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  Headache;

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  Dizziness;

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  Blurred or double vision;

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  Nausea; and

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  Mood disturbances and irritability.

These symptoms are associated with buildup of pressure in the brain as the tumor grows.

ICD-9: 191.5

ICD-10: C71.5

Choroid Plexus Carcinoma tumors spread aggressively and prognosis is generally poor. Only about one in four children will survive five years past initial diagnosis, although children who successfully undergo complete tumor resection surgery have a much more favorable outlook, with a five-year survival rate of about 60%.

Surgical intervention to remove the tumor is the preferred initial course of treatment of Choroid Plexus Carcinoma when possible. However, complete surgical resection often presents high risk of damage to nearby areas of the brain. Partial resection is commonly employed in combination with other treatment modalities including chemotherapy and radiation to mitigate risk to the patient. Follow-up surgery may be necessary in the event of recurrence.

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;

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  Operative reports; and

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  Pathology/biopsy reports.

Suggested Listings for Evaluation:

REMARKS

Treatment of Choroid Plexus Carcinoma is often surgery followed by chemotherapy radiation therapy or both, which would satisfy listing 113.13 C.