Choroid Plexus Tumor

What is choroid plexus tumor?

A choroid plexus tumor is a rare tumor that starts in the brain’s ventricles. Ventricles are spaces in the brain that are filled with cerebrospinal fluid. 

Choroid plexus tumors can be either benign (not cancer) or malignant (cancer). They are most common in infants but can occur at any age. These tumors make up about 3% of brain tumors in children under age 15. In the United States, 75–80 children and young adults under age 21 are diagnosed with choroid plexus tumors each year.  

Surgery is the most common treatment for choroid plexus tumor. Some children may also need chemotherapy, radiation therapy, or both to get rid of cancer cells that remain after surgery. 

Symptoms of choroid plexus tumor

Choroid plexus tumor symptoms vary based on the child’s age and tumor location.

As the tumor grows, it can block the flow of cerebrospinal fluid, causing fluid to build up in the brain. This is called hydrocephalus. When fluid builds up, it puts pressure on the brain (intracranial pressure). This pressure causes many of the tumor’s symptoms.

Signs and symptoms of choroid plexus tumor may include:  

• Headache
  • Often worse in the morning
  • Can improve after vomiting
• Nausea and vomiting
• Unusual sleepiness  
• Problems with balance or walking 
• Changes in vision
• Seizures
• Change in behavior or irritability
• In infants, an increase in head size (due to hydrocephalus) or fullness of the fontanel (“soft spot” at the top of the skull)

Risk factors for choroid plexus tumor

Children with Li-Fraumeni syndrome (LFS) are at higher risk for a type of choroid plexus tumor called choroid plexus carcinoma. LFS is often inherited, meaning it runs in families. But some children develop LFS without a family history.

Diagnosis of choroid plexus tumor

Tests to diagnose choroid plexus tumor include:

• A physical exam and health history 
• A biopsy involves removing a small sample of the tumor during surgery. A pathologist looks at the tissue sample under a microscope. 
• Molecular testing of the sample. Health care teams use genetic tests to look for changes in the tumor’s genes. These tests help doctors confirm the diagnosis, plan treatment, and predict the prognosis. Molecular testing is required to get a correct diagnosis. 
• A neurological exam to look at the function of the brain, spinal cord, and nerves. These tests include measures of memory, vision, hearing, muscle strength, balance, coordination, and reflexes. 
• Imaging tests, including magnetic resonance imaging (MRI) and computed tomography (CT) to provide detailed views of the brain. 
• A lumbar puncture to look for cancer cells in the cerebrospinal fluid.

Types of choroid plexus tumors

Types of choroid plexus tumor include:

• Choroid plexus papilloma (CPP) is the most common. About 80% (8 in 10) of choroid plexus tumors are CPPs. These are noncancerous and grow slowly. They rarely spread.
• Atypical choroid plexus papilloma (aCPP) is not considered fully cancerous, but it grows faster and behaves more aggressively than CPP.
• Choroid plexus carcinoma (CPC) is a cancerous form of choroid plexus tumor. It grows faster. It is more likely to spread through the cerebrospinal fluid to other tissues. About 10–20% of choroid plexus tumors are CPCs. Most choroid plexus carcinomas occur in infants and children younger than 5 years old.

Grading and staging of choroid plexus tumor

Choroid plexus tumors may be benign or malignant. Tumors can be grouped by how they look under the microscope. The tumor’s grade is based on how abnormal the cancer cells look under a microscope. The more abnormal cells look, the higher the grade. Higher-grade tumors are more likely to grow quickly and may need more aggressive treatment. 

• Choroid plexus papilloma tumors are grade 1. 
• Atypical choroid plexus papilloma tumors are grade 2. 
• Choroid plexus carcinoma is grade 3.

The stage shows how far the cancer has spread. Staging includes imaging tests like MRI scans of the brain and spine to check for tumor spread, as well as a lumbar puncture to look for cancer cells in the cerebrospinal fluid. 

Treatment of choroid plexus tumors

The main treatment for a choroid plexus tumor is surgery. In some cases, treatment may include chemotherapy, radiation therapy, or both. 

Treatment depends on:

• The type of tumor
• The tumor’s size and location  
• The child’s age

Children with choroid plexus papilloma may not need more treatment if the tumor is removed with surgery.

Children with choroid plexus carcinoma usually need chemotherapy and radiation along with surgery.

Surgery for choroid plexus tumors

Surgery is used to both diagnose and treat choroid plexus tumor. The goal of surgery is to remove as much of the tumor as possible. In rare cases, surgery may not be possible because of the tumor’s location.

In some cases, a shunt is placed in the brain to keep fluid from building up (hydrocephalus). This fluid causes increased intracranial pressure and may cause many of the tumor’s symptoms.

A shunt is a small tube that removes extra fluid from the brain to reduce symptoms. It may be temporary or permanent.

Chemotherapy for choroid plexus tumors

Chemotherapy is often used along with surgery to kill cancer cells that remain after surgery. Chemotherapy drugs used to treat choroid plexus tumors may include ifosfamide, carboplatin, etoposide, vincristine, and cyclophosphamide.

Radiation therapy for choroid plexus tumors

Radiation therapy is sometimes used along with other treatments. The type of radiation therapy used depends on the location of the tumor and if it has spread. The child’s age is also an important consideration in the use of radiation.

Clinical trials for choroid plexus tumors

Children with relapsed choroid plexus tumors who have not fully responded to chemotherapy and radiation may be offered treatment in a clinical trial. There are no specific drugs for choroid plexus carcinoma yet. But patients may be able to join immunotherapy or early-stage clinical trials. 

Supportive therapy for choroid plexus tumors

Supportive care for patients with choroid plexus tumors includes rehabilitation therapy and neurological care. Steroid and anti-seizure medications may be needed.

More support can address issues in learning, developmental milestones, and coping with cancer.

Prognosis for choroid plexus tumor

The outlook or chance of cure for choroid plexus tumors varies depending on the type and stage of the tumor:

• Choroid plexus papilloma: Generally, CPP has a very good prognosis. The survival rate is almost 100% if the tumor can be completely removed with surgery.
• Atypical choroid plexus papilloma: Prognosis for atypical CPP depends on how much tumor could be removed and how the disease responds to treatments.
• Choroid plexus carcinoma: CPC is more aggressive and grows and spreads more quickly. The chance of survival ranges from 50–70%, depending on factors like how much tumor could be removed and whether the cancer has spread.

Early diagnosis and treatment are crucial for improving outcomes for patients with choroid plexus tumors. Factors that influence prognosis include:

• Age of the child. Children under age 4 have a poorer prognosis.
• If cancer spreads to other areas of the brain and spinal cord
• How much of the tumor remains after surgery. Complete removal improves the chances of survival.
• If there are certain inherited syndromes such as Li-Fraumeni syndrome
• The use of radiation to treat the tumor if the child’s age allows it. 
• The presence of TP53 mutations in the tumor
  • The chance of survival decreases with the presence of TP53 gene mutations in the tumor.
  • Children with 2 copies of the mutated (changed) TP53 gene may have a lower chance of recovery, so early monitoring and aggressive treatment are especially important.

Support for patients with choroid plexus tumor

Long-term survivors of choroid plexus tumor need close and prolonged monitoring for the late effects of the treatment.

If your child has a TP53 gene mutation or Li-Fraumeni syndrome, ask your care team about genetic counseling. Children also need to be watched for the development of other cancers that may happen along with this disease.

Questions to ask your care team

• What type of choroid plexus tumor does my child have?
• Has the tumor spread beyond its original location?
• Is surgery an option? If so, how much of the tumor can likely be removed?
• Will my child need chemotherapy or radiation therapy? 
• Are clinical trials available for my child?
• Will my child need a shunt to treat hydrocephalus?
• Should we consider genetic counseling for our family?
• What is my child’s prognosis based on their tumor type and treatment plan?
• What follow-up care or long-term monitoring will be needed?

Find more information

Related content