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About Childhood Choroid Plexus Tumor

  • Choroid plexus tumors occur in the brain within areas called the ventricles. The ventricles contain cerebrospinal fluid that surrounds the brain and spinal cord.

    • Choroid plexus tumors are rare, representing only 3 percent of brain tumors in children.
    • They account for 10 to 20 percent of brain tumors in children under 1 year old.
    • Girls and boys are equally affected.
    • The majority of choroid plexus tumors are choroid plexus papilloma or choroid plexus carcinoma. Choroid plexus papilloma is often easier to treat.
    • Li-Fraumeni syndrome, a rare genetic condition, is associated with choroid plexus carcinoma. If your child has choroid plexus carcinoma, their doctor will discuss genetic testing for Li-Fraumeni syndrome.

    At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, we offer advanced and compassionate care for children with choroid plexus tumors. Our Brain Tumor Center specialists have extensive expertise in diagnosing and treating rare brain tumors.

    Symptoms of Childhood Choroid Plexus Tumor

    Choroid plexus tumor symptoms result from increased pressure in the brain. They may include:

    • Headache, generally upon awakening in the morning
    • Irritability, particularly in younger children who can't express where they have pain
    • Increase in head size (seen in infants)
    • Hydrocephalus, a blockage of the normal flow of cerebrospinal fluid; pressure can increase in the brain, and the skull can enlarge
    • Nausea and vomiting, often worse in the morning and improve throughout the day
    • Lethargy
    • Problems feeding or walking
    • Enlarged fontanelles, which are the "soft spots" between the skull bones in infants

    These symptoms may resemble those of other medical problems. It’s essential to consult your child's physician for a diagnosis if you witness any of these symptoms.

    How We Diagnose and Classify Childhood Choroid Plexus Tumor

    Your child’s doctor will order various diagnostic tests to confirm a choroid plexus tumor diagnosis. These tests may include physical and neurological exams, imaging studies, lumbar puncture, and biopsy.

    After completing all necessary tests, our experts meet to review the results and identify the choroid plexus tumor type.

    Treatment options will depend on the tumor type. There are three main types:

    • Choroid plexus papilloma: It is the most common type of and account for about 80 percent of childhood choroid plexus tumors. It grows slowly and rarely spreads.
    • Atypical plexus papilloma: It is the least common type. It also grows slowly and rarely spreads. However, it can sometimes act more aggressively than choroid plexus papilloma.
    • Choroid plexus carcinoma: It accounts for between 10 to 20 percent of all childhood choroid plexus tumors. It grows aggressively and is more likely to spread.

    Your doctor will share the diagnostic findings and outline treatment options with you and your family.

    How We Treat Childhood Choroid Plexus Tumor

    Your child's physician will determine a specific course of treatment based on several factors, including your child's age and medical history, as well as the tumor’s characteristics.

    Choroid plexus tumor treatment may include:

    • Surgery: Children with choroid plexus papilloma experience excellent results from surgery. Surgery can typically completely remove the tumor with no further treatment needed in between 85 to 100 percent of children.
    • In children with choroid plexus carcinoma, the goal is to remove as much of the tumor as possible. However, they will typically need additional treatment.

    • Chemotherapy: Chemotherapy treatment involves medications that interfere with a cancer cell’s ability to grow or reproduce. Doctors use it to shrink tumors and destroy remaining cancer cells. Different groups of chemotherapy drugs work in different ways. They are generally systemic treatments. Your child may receive chemotherapy in the following ways:
      • Orally, as a pill to swallow
      • Intramuscularly, as an injection into the muscle or under the skin
      • Intravenously (IV), as a direct injection into the bloodstream
      • Intrathecally, as a direct injection into the spinal fluid
    • Radiation therapy: Doctors may use radiation to control the local growth of a tumor. Radiation therapy involves using high-energy waves to damage or shrink tumors. Due to the potential side effects of radiation, including impacts on learning and hormone function, we typically avoid this treatment for young children, especially those under 3 years old.

    Ongoing care and support are also available through our extensive pediatric cancer survivorship programs, including the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic for pediatric brain tumor survivors, which provides access to same-day follow-up visits with your child’s care team and access to support specialists, including speech, physical, occupational, and integrative therapy specialists.

    Research and Clinical Trials for Childhood Choroid Plexus Tumor

    For many children with brain tumors or other rare or hard-to-treat conditions, clinical trials provide new options.

    Our research scientists continue to test new types of therapies for patients with brain tumors through clinical trials. If you have any questions about available clinical trials, your child’s medical team can provide you with additional information.

    In addition to launching our own clinical trials, Dana-Farber/Boston Children’s also offers trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Pediatric Neuro-Oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group.

    Long-term Outcomes for Children with Choroid Plexus Tumor

    Children with choroid plexus papilloma do exceptionally well with surgery. The five-year survival following complete surgical removal is 80 to 100 percent. Partial surgical removal results in a 70 percent five-year survival rate.

    Children with choroid plexus carcinoma typically need additional treatment after surgery. The survival rate after surgery is 60 to 65 percent.

    There are several standard and experimental treatment options for children with progressive or recurrent choroid plexus tumors. A second surgery may be necessary for recurrent tumors, followed by either chemotherapy or radiation therapy.

    Childhood Choroid Plexus Tumor Treatment Team

    Our brain tumor treatment team includes specialists dedicated to treating children with conditions such as choroid plexus tumors. See a complete list of the specialists in our Childhood Brain Tumor Center.