Choroid Plexus Tumor

What is choroid plexus tumor?

Choroid plexus tumors are rare tumors that start in the brain’s ventricles. Ventricles are spaces in the brain that fill with cerebrospinal fluid.

Choroid plexus tumors can be either benign or malignant.

How common are choroid plexus tumors?

Every year 75 to 80 children and young adults under 21 in the U.S. are diagnosed with a choroid plexus tumor. They are most common in infants. But people can develop choroid plexus tumors at any age.

What are the types of choroid plexus tumors?

There are 2 main types of choroid plexus tumor:

• Choroid plexus papilloma (CPP) is the most common. About 80% of choroid plexus tumors are CPPs. These are noncancerous and grow slowly. They rarely spread.
• 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 CPC.

Most choroid plexus carcinomas occur in infants and children younger than 5. Increased risk for choroid plexus carcinoma is associated with Li-Fraumeni syndrome, an inherited syndrome.

What is the prognosis for choroid plexus tumors?

With complete tumor removal, choroid plexus papilloma (CPP) survival rates are nearly 100%. Choroid plexus carcinoma (CPC) is more aggressive. But the chance of cure is about 50-70%.

Factors that influence prognosis include:

• Children with CPP have a better prognosis compared to children with CPC
• Age of the child (children under 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 chances of survival)
• If there are certain inherited syndromes (e.g., Li-Fraumeni or germline TP53 mutation) 
• The use of radiation to treat the tumor
• 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 gene in their tumor have an extremely poor prognosis.

How are choroid plexus tumors treated?

The main treatment for a choroid plexus tumor is surgery.

In some cases, doctors recommend more treatments like chemotherapy and radiation therapy. Treatment needs vary depending on:

• The type of tumor
• The extent of the disease after surgery

Signs and symptoms of choroid plexus tumor

Symptoms vary based on the child’s age and tumor location.

As the tumor grows, cerebrospinal fluid is blocked and can build up in the brain. This is called hydrocephalus. The fluid causes the ventricles to widen. It increases pressure on the brain (intracranial pressure).

Increased pressure against brain tissue causes many of the symptoms.

Choroid plexus tumor symptoms may include:

• Headache
  • Often worse in the morning
  • Can improve after vomiting
• Nausea and vomiting
• Changes in activity levels
• Lethargy
• Difficulty walking 
• Problems eating
• Change in behavior or irritability
• Increased head size in infants
• Increase of fullness of the fontanel (“soft spot” at the top of the skull)

Diagnosis of choroid plexus tumor

Doctors test for choroid plexus tumors in several ways. These tests include:

• A biopsy is performed to diagnose CPT. In a biopsy, a small sample of the tumor is removed during surgery using a needle. A pathologist looks at the tissue sample under a microscope. She can identify the specific type of tumor.
• A health history and physical exam help doctors learn about:
  • Symptoms
  • General health
  • Past illness
  • Risk factors
  • Developmental milestones
• A neurological examination to look at:
  • Brain function
  • Spinal cord
  • Nerves
  • Tests measuring:
    • Memory
    • Vision
    • Hearing
    • Muscle strength
    • Balance
    • Coordination
    • Reflexes
• Imaging tests including magnetic resonance imaging (MRI) and computerized tomography (CT)
  • These create detailed pictures of the ventricles, brain, and spinal cord.
  • Doctors can see the size and location of the tumor.
• A lumbar puncture looks for cancer cells in the cerebrospinal fluid.

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. Staging involves an MRI of the spine and brain and a lumbar puncture. The more abnormal cells look, the higher the grade.

Here is how they can be grouped:

• Choroid plexus papilloma
  • Generally, not cancerous
  • Usually Grade I
• Choroid plexus carcinoma
  • Generally, cancerous
  • Typically Grade III
  • More aggressive
  • Can grow rapidly

How are choroid plexus tumors treated?

Treatment for choroid plexus tumors may include surgery, chemotherapy, and radiation.

Choroid plexus papillomas and atypical choroid plexuses tumors are managed with surgery when possible.

Choroid plexus carcinoma is a more aggressive cancer. Most of these patients will receive many types of treatments.

Treatment depends on several factors:

• Size and location of the tumor
• The child’s age
• The type of tumor

Life after choroid plexus tumor

Children who are long-term survivors of CPT need close and prolonged monitoring for late effects of the treatment.

Children with a germline TP53 mutation or Li-Fraumeni syndrome need a referral for genetic counseling. They also need surveillance for other associated cancers.

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