Neurosurgical Consultants: Nervous System Tumors - Benign: Choroid Plexus Papilloma

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CHOROID PLEXUS PAPILLOMA

CHOROID PLEXUS PAPILLOMA is a rare, benign tumor most common in children younger than 12 although they are encountered in adults as well. The Choroid Plexus (the structure from which Choroid Plexus Papillomas arise) is a normal anatomical structure that lies within the Ventricular System of the Brain and is the source of manufacture of Cerebrospinal Fluid (CSF).

Figure 1: Diagram of the Cerebrospinal Fluid Pathway. The Ventricular locations of the Choroid Plexus apparatus are demonstrated in each of the Lateral Ventricles as well as the 3rd & 4th Ventricles.

Choroid Plexus Papillomas constitute less than 1% of Brain tumors in patients of all ages. They account for 3% of childhood intracranial neoplasms with a propensity for younger ages. They comprise 5% of the intracranial neoplasms in children younger than 2 years and 12% of intracranial neoplasms in children younger than 1 year of age.

Choroid Plexus Papillomas have been associated with von Hippel-Lindau syndrome. Males tend to be affected by these tumors by a factor of almost 3:1 compared to females.

Choroid Plexus Papillomas generally grow slowly until they eventually block the flow of Cerebrospinal Fluid causing Hydrocephalus and increased intracranial pressure. Symptoms can also be produced in these cases by the hyper-secretion of CSF by tumor cells which can lead to an increased amount of fluid and, eventually, to Hydrocephalus. In either case, eventual progression and increased intracranial pressure can occur. Cases of Hydrocephalus occasionally do not resolve despite complete surgical resection, possibly as a result of some disturbance of the CSF reabsorbtion mechanisms or blockage at other sites within the Ventricular system (See Figure 1 for the CSF pathways).

SYMPTOMS

slowly increasing signs of mental dysfunction
new onset seizures
persistent headaches
persistent nausea & vomiting suggesting increased intracranial pressure
visual deterioration
drowsiness

diplopia (double vision resulting from 3rd and/or 6th Cranial Nerve deficits)

DIAGNOSIS

Physical examination may disclose papilledema, visual field abnormalities, and Oculomotor deficits (involving the 3^rd, 4^th and/or 6^th Cranial Nerves).

MRI Scan is usually diagnostic when it demonstrates a large, densely "enhancing" intraventricular tumor in proximity to the Choroid Plexus. Among the tumors of the Choroid Plexus to be considered in the differential diagnosis of lesions in this location are metastatic deposits (cancerous tumors from lung, breast, kidney, etc) Meningiomas as well as Glial tumors.

Figure 2A (Left): MRI Scan (Gadolinium Enhanced Axial View) A large Right Lateral Ventricle Choroid Plexus Papilloma with Hydrocephalus (enlarged Ventricles indicated by the Quadrangular Arrows) which results from the "Hypersecretion" of CSF by the tumor.

Figure 2B (Right): MRI Scan (Gadolinium Enhanced Coronal View) The size, shape, configuration and pattern of "enhancement" is characteristic for a Choroid Plexus Papilloma in this young person.

TREATMENT

Surgery

Choroid Plexus Papillomas can often be entirely removed surgically resulting in a complete cure. Ventricular shunt insertion should be avoided as an alternative to direct surgical removal. In some urgent circumstances a Ventriculostomy (the insertion of a temporary drain within a Ventricle) may be required in order to establish control of critically increased intracranial pressure. The success of surgical resection usually is evident soon after the operation. Follow-up is required including sequential MRI Scans to evaluate Ventricular size as well as the effectiveness of the resection. Since recurrence of the tumor is possible, long term follow up MRI Scans are recommended.

MALIGNANT TRANSFORMATION

Choroid Plexus Carcinoma is a rare, malignant form of this Choroid Plexus Papilloma. This malignant variety is not curable with surgery alone and invariably requires additional therapy including Chemotherapy and Radiation Therapy.

Since Choroid Plexus Carcinomas exist within the Ventricular system and are in direct constant contact with the Cerebrospinal Fluid, they have a tendency to "seed" (spread) through the entire Cranio-spinal axis. In these cases Radiation Therapy of the entire Brain and Spinal Canal is required.