Choroid plexus papilloma, also known as papilloma of choroid plexus, is a rare benign neuroepithelial intraventricular WHO grade I lesion found in the choroid plexus. It leads to increased cerebrospinal fluid production, thus causing increased intracranial pressure and hydrocephalus.
Choroid plexus papilloma occurs in the lateral ventricles of children and in the fourth ventricle of adults. This is unlike most other pediatric tumors and adult tumors, in which the locations of the tumors is reversed. In children, brain tumors are usually found in the infratentorial region and the in adults, brain tumors are usually found in the supratentorial space. The relationship is reversed for choroid plexus papillomas.
The tumor is neuroectodermal in origin and similar in structure to a normal choroid plexus. They may be created by epithelial cells of the choroid plexus.
Frequency and age affected
Internationally tumors of the choroid plexus are rare, accounting for 0.4-0.6% of all intracranial neoplasms. It most commonly affects young children under the age of 5 with a mean patient age of 5.2 years.
Signs and symptoms
Signs of the tumor resulting from increased intracranial pressure are present in 91% of patients, with vomiting, homonymous visual field defects and headache being the most common symptoms.
Other symptoms are ear ringing and dizzyness.
Choroid plexus papillomas are benign tumors that are usually cured by surgery; malignant progression has been rarely reported.
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