Choroid Plexus Brain Tumor

Choroid plexus brain tumors develop in the tissue located in the spaces of the brain called ventricles. This tissue makes cerebrospinal fluid (CSF), which surrounds the brain and spinal cord. These rare tumors are seen more often in younger children. Between 10 and 20 percent of brain tumors that occur within the first year of life are choroid plexus tumors.

Successfully treating your child’s brain tumor depends on what type of choroid plexus tumor your child has. Doctors at Dana-Farber/Boston Children's will classify your child's tumor as one of the following types:

• Choroid plexus papillomas (CPPs): These are one of two types of choroid plexus tumor that grow slowly, rarely spread and are by far more common.
• Atypical plexus papillomas (APPs): Like CPPs, these tumors grow more slowly than their aggressive counterparts and rarely spread.
• Choroid plexus carcinomas (CPCs) grow more aggressively and are more likely to spread than either APPs or CPPs. CPCs are less common, accounting for 25-35 percent of all choroid plexus tumors.

Choroid plexus papillomas are often easier to treat than carcinomas. An APP can act more aggressive but usually behaves like a CPP.

Brain tumors can cause a variety of symptoms in children depending on their size and location. Keep in mind that the symptoms of a brain tumor may resemble other more common conditions or medical problems. It is important to consult your child's physician for a diagnosis.

Choroid plexus tumors symptoms are most often associated with increased pressure in the brain and can include:

• Headache, generally upon awakening in the morning
• Irritability, particularly in younger children who can’t express where they have pain
• Increase in head size (seen in infants)
• Hydrocephalus: blockage of the normal flow of cerebrospinal fluid; pressure can increase in the brain and the skull can enlarge
• Nausea and vomiting. These symptoms are often worse in the morning and improve throughout the day
• Lethargy
• Problems feeding or walking
• Enlarged fontanelles, which are the “soft spots” between the skull bones in infants

As a parent, you undoubtedly want to know what may have caused your child’s tumor. It’s important to understand that these and other brain tumors most often occur with no known cause. There’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

One rare genetic disease called Li-Fraumeni syndrome can be associated with choroid plexus tumors. If your child has a choroid plexus tumor their doctors will talk to you about genetic testing for Li-Fraumeni syndrome.

The first step in treating your child is forming an accurate and complete diagnosis. Choroid plexus tumors are most commonly diagnosed from imaging studies and biopsy.

Choroid plexus tumor diagnostic tests may include:

• A physical exam and complete medical history
• A neurological function test of reflexes, muscle strength, eye and mouth movement, coordination, and alertness
• Magnetic resonance imaging (MRI)
• Lumbar puncture (spinal tap)

There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.

Your child's physician will determine a specific course of treatment based on several factors, including your child's age, medical history, the type, location, and size of the tumor and the extent of the disease.

Choroid plexus tumor treatment may include:

• Neurosurgery to remove the tumor
• Chemotherapy
• Radiation

Patients with choroid plexus papilloma do extremely well with surgery. Children with choroid plexus carcinoma have a more guarded prognosis and often require aggressive treatment.

The five-year survival rate for children with CPP is 80 to 100 percent following complete surgical removal of the tumor and about 70 percent with partial removal. CPC requires additional postoperative treatment, with a survival rate of about 60 to 65 percent.

There are a number of standard and experimental treatment options for children with progressive or recurrent choroid plexus tumors. A second surgery may be necessary for recurrent tumors, followed by either chemotherapy or radiation therapy.

Children with choroid plexus tumors are treated through the Brain Tumor Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors. Our brain tumor specialists have extensive expertise in treating all types of neural tumors, including choroid plexus tumors.