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).
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
Physical examination may disclose papilledema, visual field
abnormalities, and Oculomotor deficits (involving the 3rd,
4th and/or 6th Cranial Nerves).
- slowly increasing signs of mental dysfunction
- new onset seizures
- persistent headaches
- persistent nausea & vomiting suggesting increased intracranial pressure
- visual deterioration
- diplopia (double vision resulting from 3rd and/or 6th Cranial Nerve deficits)
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
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.
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.
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This page last edited on 2/19