Choroid plexus carcinoma

Date Updated: 08/18/2020

Overview

A choroid plexus carcinoma is a rare cancerous (malignant) brain tumor that occurs mainly in children.

A choroid plexus carcinoma begins near the brain tissue that secretes cerebrospinal fluid. A noncancerous tumor of this area is called a choroid plexus papilloma. As the tumor grows, it can affect the function of nearby structures in the brain, causing excess fluid in the brain (hydrocephalus), irritability, nausea or vomiting, and headaches.

Treatment and chance of recovery (prognosis) depend on the tumor's size, location, whether it has spread, and your child's age and general health.

Diagnosis

A choroid plexus carcinoma most often occurs in children under 2 years old. Tests and procedures used to diagnose choroid plexus carcinoma include:

Physical exam. Diagnosing a choroid plexus carcinoma usually starts with a medical history review and a neurological exam. During this procedure, your child's vision, hearing, balance, coordination and reflexes are tested.
Brain imaging tests. Tests to create images of your brain may include magnetic resonance imaging (MRI) and computerized tomography (CT). An MRI also helps your doctor to plan the surgery.
Genetic tests. Tests to identify genetic aspects of some choroid plexus carcinomas are available. Ask your doctor about genetic counseling.

Treatment in children usually differs from treatment in adults. If your child receives a diagnosis of choroid plexus carcinoma, ask your doctor to refer you to a specialist in treating children with brain tumors (pediatric neuro-oncologist, pediatric radiation oncologist).

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 the entire tumor, when possible. But because delicate and important structures may be nearby, doctors sometimes can't remove the entire tumor. Generally, people who undergo surgery for a choroid plexus carcinoma will need additional therapy after surgery.

After surgery, about half of the people who had excess fluid on the brain (hydrocephalus) experience relief from this symptom. Others may need to have a temporary drain (shunt) placed at the surgical site.
Chemotherapy. Chemotherapy may be used in addition to surgery and radiation therapy to help control the tumor.
Radiation therapy. Radiation may be used after surgery, even if the entire tumor was successfully removed. Radiation may also be used later if the tumor grows back.

Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill tumor cells. Advanced technologies such as stereotactic radiosurgery, proton therapy, intensity-modulated radiation therapy (IMRT) and intraoperative radiation therapy (IORT) help treat the cancer effectively while sparing healthy tissues, such as the eyes, optic nerves, brain, brainstem and spinal cord.
Clinical trials. Clinical trials are studies of new treatments or new ways of using existing treatments.