• Choroid Plexus Tumor Overview

    Choroid plexus tumors arise in the tissue located in the spaces of the brain called ventricles. This tissue makes cerebrospinal fluid (CSF), which surrounds the brain and spinal cord. These rare tumors are seen more often in younger children. Between 10 and 20 percent of brain tumors that occur within the first year of life are choroid plexus tumors.

    Choroid Plexus Tumor Treatment at Dana-Farber/Boston Children's

    Children with choroid plexus tumors are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Brain Tumor Center. Continue reading to learn more about choroid plexus tumors or visit the Brain Tumor Center homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    There are three main types of choroid plexus tumors:

    • Choroid plexus papillomas (CPP) and atypical papillomas (APP) grow more slowly, rarely spread , and are by far more common
    • Choroid plexus carcinomas (CPC) grow more aggressively and are more likely to spread

    A choroid plexus tumor can block the normal flow of CSF, causing increased pressure on the brain (hydrocephalus) and enlargement of the skull. It can also cause symptoms including:

    • Headache – generally upon awakening in the morning
    • Nausea and vomiting – often worse in the morning and improving throughout the day
    • Lethargy
    • Irritability
    • Problems feeding or walking
    • Enlarged fontanelles (the “soft spots” between the skull bones in infants)

    In addition to a complete medical history, physical examination and neurological exam, which tests reflexes, muscle strength, eye and mouth movement, and coordination and alertness, choroid plexus tumors are diagnosed with:

    • Magnetic resonance imaging
    • Lumbar puncture (spinal tap)

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Doctors recommend a number of treatments for choroid plexus tumors, including:

    • Neurosurgery – 80 to 100 percent of children with CPP are treated successfully with complete removal of the tumor
    • Chemotherapy – either before surgery to shrink the tumor or to kill cancer cells
    • Radiation therapy – to kill any cancer cells remaining after surgery; rarely used in children under 3 because of potential effects on learning and hormone function

    Progressive or Recurrent Disease

    There are a number of standard and experimental treatment options for children with progressive or recurrent choroid plexus tumors. A second surgery is common for recurrent tumors, followed by either chemotherapy or radiation therapy.

    Long-term Outlook

    Patients with CPP do extremely well with surgery. Patients with CPC have a more guarded prognosis and often require aggressive treatment. The five-year survival rate for children with CPP is 80 to 100 percent following complete surgical removal of the tumor and about 70 percent with partial removal.  In most cases, CPC requires additional postoperative treatment, with a survival rate of about 65 percent.