Choroid Plexus Papilloma

Choroid plexus papilloma (CPP) is a rare type of brain tumor. They are generally benign (not cancer). Benign also means that the growth does not spread to other parts of the body. CPP is most common in young children, but it can also appear in adults. CPP are generally slow-growing tumors. It is very rare for this type of tumor to spread outside the brain. In atypical CPP, the tumor is more likely to grow faster or come back after treatment.

A CPP tumor grows in the choroid plexus. This is a layer of tissue within the brain that makes the cerebrospinal fluid (CSF). CSF surrounds and cushions the brain and spinal cord. The choroid plexus lines the ventricles of your brain, which store the CSF. The choroid plexus is made of connective tissue, blood vessels, and the ependymal cells.

In adults, CPP tends to form in the fourth ventricle, while in children it forms on the lateral ventricle. The fourth ventricle is in between the cerebellum and the brainstem. The cerebellum is the part of your brain that controls your balance and coordination. The brainstem controls your breathing, swallowing, eye movements, and other vital functions.

CPP may cause your brain to make too much CSF. It might also block the normal drainage of CSF. This excess CSF in the brain (hydrocephalus) can create pressure in the brain that can be dangerous.

Even if a CPP is not cancer, it can still cause problems. It may press against brain tissue and cause symptoms, such as headache, vision problems, or nausea and vomiting. The standard treatment for CPP is surgery to remove the tumor.

Healthcare providers do not yet know what causes CPP. It is possible that certain infections, genetic factors, immune system problems, or environmental factors play a role in causing these tumors. In some cases, CPP may be linked with certain rare gene changes (mutations).

CPP most often leads to increased pressure in the brain. This can cause symptoms such as:

• Headache (common).
• Nausea and vomiting.
• Sleepiness.
• Eye problems (secondary to cranial nerve deficits).
• Dizziness and balance problems.
• Difficulty walking.
• Seizures (rare).
• Psychosis (rare).
• Changes in hearing.

Your symptoms may be different, depending on the location of the CPP tumor in your brain. Your symptoms may start slowly and get worse over time.

Your primary healthcare provider may refer you to a neurologist. This is a healthcare provider who specializes in diagnosing and treating diseases of the central nervous system. You may see a neuro-oncologist. This is a healthcare provider who specializes in cancers of the brain and spinal cord. You may be referred to a neurosurgeon. This is a surgeon who performs brain or spinal cord surgery.

The process starts with a medical history and a physical exam. Your healthcare provider will ask about your symptoms and past medical conditions. He or she may also ask about your family’s medical history. The physical exam may include a neurologic exam. During a neurologic exam, your healthcare provider may ask you to do things like walk, touch your finger to your nose, or hold your hands out. He or she may look in your eyes with a light. Tests may be done, such as:                                    

• CT scan. This is an imaging test that uses X-rays and a computer to make detailed images of the body. Scans may be done of your brain.
• MRI. This test uses large magnets and a computer to create images of the body. MRI scans of your brain may be done to get more information. Contrast dye may be used to help show more detail in the images.   
• Biopsy. Small pieces of tissue are taken from the tumor. They are looked at with a microscope in a lab.

The main treatment for CPP is surgery. Complete removal of the tumor with surgery usually cures CPP. These tumors rarely come back. In most cases, patients with CPP have a good outcome after surgery.

If a CPP tumor is only partly removed, it may grow again, but it likely will not spread. An atypical CPP is more likely to come back after treatment. If CPP does come back, you may need chemotherapy or radiation therapy to treat it.

You will need to work with your medical team to decide on the best treatment plan for you. You will need to have ongoing follow-up with your healthcare provider. This will include repeated imaging tests to check if the tumor comes back.

Fluid in the brain (hydrocephalus) is a common complication. This is because CPP happens in the ventricles of the brain filled with CSF. You may need a tube to drain excess CSF and lower the pressure in your brain. After the tumor is removed, this problem will likely go away.

In rare cases, there may be complications from the surgery. For example, there is a very small chance of a problem with the facial nerve. This could cause paralysis of part of your face. This is a rare problem.