Choroid plexus carcinoma

Overview

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 tumor's size, location, whether it has spread, and your child's age and general health.

Diagnosis

Choroid plexus carcinoma happens the most in children under 2 years old. 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 health care 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 health care provider 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 health care provider 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

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 health care provider if you might be able to be in a clinical trial.