Choroid Plexus Tumors (Pediatric)

Choroid plexus tumors are rare growths that form in the choroid plexus, the part of the brain that makes cerebrospinal fluid. Most of these tumors — about 90 percent — are benign, meaning they are not cancer. They occur most often in children under the age of two.

As these tumors grow, they can block the normal flow of cerebrospinal fluid. This buildup, called hydrocephalus, can increase pressure on the brain and cause the skull to enlarge in young children.

Types & Stages 

Doctors classify choroid plexus tumors into three types:

• Choroid plexus papillomas (CPPs): The most common type. They grow slowly and rarely spread.
   
• Atypical choroid plexus papillomas (APPs): Also slow-growing and unlikely to spread.
   
• Choroid plexus carcinomas (CPCs): The cancerous type. They grow faster and are more likely to spread. CPCs make up about 25 to 35 percent of all choroid plexus tumors.

Signs & Symptoms

Symptoms can include:

• Headaches
• Nausea and vomiting
• Blurred or double vision
• Increased tiredness
• Irritability
• Seizures

Diagnosis

To find and understand a choroid plexus tumor, your child's care team may use:

• Neurological exam. Tests your child's vision, hearing, balance, coordination, and reflexes to see which part of the brain may be affected.
   
• Brain imaging. An MRI or CT scan creates detailed images of the brain. MRI also helps the team plan surgery.
   
• Genetic testing. Some of these tumors are linked to gene changes that run in families. Ask your child's doctor about genetic testing and counseling.

Treatment

Treatment depends on the type of tumor and may include one or more of the following:

• Surgery. The goal is to remove as much of the tumor as possible. Because important structures sit nearby, surgeons may not be able to remove every cell, so other treatments may follow. Surgery can also relieve hydrocephalus. In some cases, a small temporary tube called an external ventricular drain (EVD) is placed to drain extra fluid.
   
• Radiation therapy. May be used after surgery or later if the tumor returns.
   
• Chemotherapy. May be used along with surgery and radiation to help control cancer, sometimes at the same time as radiation.

Risk Factors

A few factors can raise the risk of a choroid plexus carcinoma:

• Age. These tumors can appear at any age but happen most often in children under two.
   
• Inherited syndromes. Some are linked to genetic conditions that run in families, such as Li-Fraumeni syndrome.

Screening

There is no routine screening test for choroid plexus tumors. Diagnosis depends on recognizing symptoms early and getting a prompt medical exam. If you notice signs in your child, talk with your doctor right away.