Choroid Plexus Carcinoma

What is choroid plexus carcinoma?

Choroid plexus carcinoma is a cancerous (malignant) brain tumor that forms in choroid plexus tissue. This tissue lines the fluid-filled spaces (ventricles) in your brain. It helps make cerebrospinal fluid, which cushions and protects your brain and spinal cord.

Choroid plexus carcinoma is also known as a grade 3 choroid plexus tumor. These fast-growing tumors often spread in cerebrospinal fluid throughout the central nervous system, making them difficult to treat.

How common is choroid plexus carcinoma?

Choroid plexus carcinoma is rare. There are three types of choroid plexus tumors and, in general, they account for less than 1% of all brain tumors. Of the three types of choroid plexus tumors, choroid plexus carcinomas are the rarest and the only kind that’s cancerous. These tumors are more likely to affect infants and children.

What are choroid plexus carcinoma symptoms?

Signs and symptoms of choroid plexus carcinoma vary depending on the size and location of the tumor, but may include:

• Extreme fatigue or sleepiness (lethargy).
• Headaches.
• Irritability or fussiness.
• Nausea and vomiting.
• Numbness or weakness.
• Seizures.
• Vision problems like blurred or double vision (diplopia).

What causes choroid plexus carcinoma?

Medical experts aren’t sure why some people develop choroid plexus carcinoma. Cancers, in general, occur when a gene changes or mutates. This change causes diseased cells to rapidly grow and spread.

Risk factors

Anyone can develop choroid plexus carcinoma. But these factors may increase your risk:

• Aicardi syndrome: Children born with Aicardi syndrome have unusual brain changes that may increase their risk of choroid plexus tumors. An unknown gene change (mutation) causes this condition. It isn’t a condition passed down through families (inherited).
• Li-Fraumeni syndrome: People who have a rare genetic condition called Li-Fraumeni syndrome are more prone to developing cancers. Most people inherit a gene change from a biological parent that causes this condition.
• Biological sex: For unknown reasons, women are slightly more at risk.

How do healthcare providers diagnose choroid plexus carcinoma?

Your healthcare provider will evaluate your symptoms and perform different tests to diagnose choroid plexus carcinoma.

Depending on your age or your child’s age, these tests may include:

• Cognitive test to assess brain function.
• Neurological exam to check vision, hearing, balance issues, reflexes and coordination.
• Brain MRI or other imaging tests to look for tumors and signs of hydrocephalus.
• DNA test (genetic test) to look for gene changes linked to conditions like Li-Fraumeni or Aicardi syndrome.
• Spinal tap (lumbar puncture) to examine cerebrospinal fluid for cancer cells.

How is choroid plexus carcinoma treated?

Neurosurgeons perform brain surgery to remove cancerous and noncancerous (benign) choroid plexus tumors. After surgery, a lab examines tissue samples from the tumor (biopsy) to check for cancer cells.

If a biopsy indicates choroid plexus carcinoma, you or your child may also need:

• Chemotherapy.
• Radiation therapy.

What complications can occur after surgery for choroid plexus carcinoma?

Common complications of brain surgery include:

• Brain bleed or swelling.
• Confusion or delirium.
• Headaches.
• Infection.
• Memory loss or speech problems.

What new treatments are in development for choroid plexus carcinoma?

You or your child may benefit from promising new therapies under development (a clinical trial). These therapies include:

• Immunotherapy drugs that help your immune system find and destroy cancer cells.
• Targeted therapy drugs that target genetic changes that cause cancer.

What is recovery like after treatment for choroid plexus carcinoma?

Your recovery, including how quickly you can return to work, school and your usual activities, depends on several factors, like:

• Your age and overall health.
• Location and number of tumors.
• Follow-up treatments like chemotherapy.

You should follow your healthcare provider’s recommendations to ensure a safe recovery. You or your child may get frequent brain imaging tests to watch the healing process.

What’s the outlook (prognosis) for someone with choroid plexus carcinoma?

The outlook (prognosis) for someone with choroid plexus carcinoma depends on:

• Age.
• Overall health.
• Whether the surgeon can remove the whole tumor or only part of it.

Your outlook also depends on whether:

• The tumor grows back after surgery (recurrence).
• The cancer spreads (metastasizes) from the original tumor to other parts of your body.

Everyone is different. So, it’s best to ask your cancer care team what to expect in your specific case.

What is the survival rate for someone with choroid plexus carcinoma?

The five-year survival rate for people with choroid plexus carcinoma is 40% to 60%. This means as many as 6 in 10 people are still alive five years after receiving the diagnosis. That rate decreases to 30% if you also have Li-Fraumeni syndrome.

When should I see my healthcare provider?

You should call your healthcare provider if you or your child experience:

• Behavioral changes, like confusion or extreme irritability or fussiness.
• Breathing problems.
• Difficulty walking.
• Seizures.
• Severe headaches.
• Vision or speech changes.

What questions should I ask?

You may want to ask your healthcare provider:

• What type of brain tumor do I or does my child have?
• What is the best treatment?
• How often do I or does my child need imaging scans?