Disease Information

Brain Tumor: Choroid Plexus Tumor

Alternate Names: Anaplastic choroid plexus papilloma

Definition

The choroid plexus papilloma is a rare, benign tumor most common in children under the age of 2. The choroid plexus carcinoma is the malignant form of this tumor. The choroid plexus carcinoma is sometimes called an anaplastic choroid plexus papilloma. In very young children, the lateral ventricles are the most common location of this tumor. Ventricles are cavities in the brain that are filled with cerebrospinal fluid, including the two lateral, third, and fourth ventricles.

The choroid plexus papilloma grows slowly within the ventricles. It eventually blocks the flow of cerebrospinal fluid, causing hydrocephalus (an abnormal increase of cerebrospinal fluid in the intracranial cavity). Headache and other symptoms of increased pressure are common. The choroid plexus carcinomas commonly grow into nearby tissue and spread widely via the cerebrospinal fluid. Hydrocephalus is often present.


Incidence

About 3 percent of the primary brain tumors in children are choroid plexus papillomas. They represent less than 1 percent of all primary brain tumors. Choroid plexus carcinomas comprise about 10 percent of all choroid plexus tumors.


Survival Rates

The prognosis for choroid plexus papilloma is excellent following a complete resection of their tumor.  No further treatment is required and long-term survival is near 100 percent. Choroid plexus carcinomas require post-operative treatment in most cases, but also do very well with a survival of 65 percent.


Treatment Strategies

For choroid plexus papillomas, surgery may be the only treatment required if the tumor is completely removed. Tumor removal relieves the hydrocephalus about half the time. A shunt is required for other patients. The role of radiation or chemotherapy is still being investigated, but might be recommended for inaccessible or partially resected tumors.

For choroid plexus carcinomas, treatment often includes surgery, chemotherapy, and radiation therapy. A second surgery might be recommended for recurrent tumors, followed by some form of radiation and/or chemotherapy.


Current Research

 

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