Choroid plexus carcinoma

Choroid plexus carcinoma is a rare type of brain cancer that happens mainly in children.

Choroid plexus carcinoma begins as a growth of cells in the part of the brain called the choroid plexus. Cells in the choroid plexus produce the fluid that surrounds and protects the brain and spinal cord. This fluid is called cerebrospinal fluid, also known as CSF. As the cancer grows, it can cause too much CSF in the brain. This can lead to symptoms such as irritability, nausea or vomiting, and headaches.

Treatment and chance of recovery depend on many factors. These include the cancer’s size, location, whether it has spread, and your child’s age and general health.

Choroid plexus carcinoma symptoms include:

• Blurred or double vision.
• Headaches.
• Increased tiredness.
• Irritability.
• Nausea.
• Seizures.
• Vomiting.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if your child has any symptoms that worry you.

It’s not clear what causes most choroid plexus carcinomas. This cancer starts as a growth of cells in the brain.

Choroid plexus carcinoma happens when cells in the brain develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.

The cancer cells form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue.

Factors that increase the risk of choroid plexus carcinoma include:

• Young age. Choroid plexus carcinoma can happen at any age. This brain cancer happens most often in children under 2 years old.
• Inherited syndromes. Some choroid plexus carcinomas are linked to certain genetic changes that can run in families, such as with Li-Fraumeni syndrome.

Tests and procedures used to diagnose choroid plexus carcinoma include:

• Neurological exam. During this exam, your child’s vision, hearing, balance, coordination and reflexes are tested. This can help show which part of the brain might be affected by the tumor.
• Brain imaging tests. Tests to create images of your child’s brain may include MRI and CT. An MRI also helps your child’s healthcare team to plan the surgery.
• Genetic tests. Some choroid plexus carcinomas are linked to certain genetic changes passed down in families. Tests to identify certain genes are available. Ask your child’s healthcare professional about genetic testing and counseling.

Treatment in children usually differs from treatment in adults. If your child receives a diagnosis of choroid plexus carcinoma, ask your healthcare professional to refer you to a specialist who cares for children with brain tumors. The management of this cancer is complex. Seek out a medical center that has experience with this cancer and can offer the latest treatment options for your child.

Treatment of a choroid plexus carcinoma is often surgery followed by chemotherapy, radiation therapy or both.

• Surgery. The goal of surgery is to remove all of the cancer, when possible. But because delicate and important structures may be nearby, surgeons sometimes can’t get all the cancer cells. Other treatments are often needed after surgery.

  Surgery can help relieve the symptoms of having too much fluid in the brain, which is also called hydrocephalus. Sometimes a temporary drain is put in during surgery to drain more fluid.

• Chemotherapy. Chemotherapy uses medicines to kill cancer cells. It may be used in addition to surgery and radiation therapy to help control the cancer. Sometimes chemotherapy is done at the same time as radiation therapy.
• Radiation therapy. Radiation therapy uses powerful energy beams to kill tumor cells. The energy can come from X-rays, protons and other sources. Advanced technologies help treat the cancer effectively while sparing healthy tissues. Radiation may be used after surgery, even if the entire tumor was successfully removed. Radiation also may be used later if the tumor grows back.
• Clinical trials. Clinical trials are studies of new treatments. These studies provide a chance to try the latest treatments. The risk of side effects might not be known. Each trial has strict requirements that each person must meet in order to be in the trial. Ask your healthcare professional if you might be able to be in a clinical trial.

Make an appointment with a doctor or other healthcare professional if your child has any symptoms that worry you. If your healthcare professional thinks your child could have a condition that affects the brain, that person might refer you to a specialist. Often this is a doctor who specializes in diseases that affect the brain and nervous system, called a neurologist.

Because appointments can be brief, it’s a good idea to be prepared. Here’s some information to help you get ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there’s anything you need to do in advance, such as restrict your child’s diet.
• Write down symptoms your child has, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements your child is taking and the doses.
• Take a family member or friend along. Sometimes it can be very hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For choroid plexus carcinoma, some basic questions to ask include:

• Does my child have choroid plexus carcinoma?
• What is the stage of my child’s choroid plexus carcinoma?
• Has the choroid plexus carcinoma spread to other parts of my child’s body?
• Will my child need more tests?
• 治療の選択肢は何ですか?
• How much does each treatment increase the chances of a cure or prolong a child’s life?
• What are the potential side effects of each treatment?
• How will each treatment affect my child’s daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should my child see a specialist?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

Don’t hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your child’s symptoms begin?
• Do your child’s symptoms happen all the time or do they come and go?
• How bad are the symptoms?
• What, if anything, seems to improve the symptoms?
• What, if anything, appears to worsen the symptoms?