• Choroid Plexus Tumor

    Choroid plexus brain 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 the Brain Tumor Center, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors. Our brain tumor specialists have extensive expertise in treating all types of neural tumors, including choroid plexus tumors. Our patients receive care from neuro-oncologists, neurosurgeons, neurologists, and pediatric subspecialists.

    How are choroid plexus tumors classified?

    Successfully treating your child’s brain tumor depends on what type of choroid plexus tumor your child has. Doctors at Dana-Farber/Boston Children's will classify your child's tumor as one of the following types:

    • Choroid plexus papillomas (CPPs): These are one of two types of choroid plexus tumor that grow slowly, rarely spread and are by far more common.
    • Atypical plexus papillomas (APPs): Like CPPs, these tumors grow more slowly than their aggressive counterparts and rarely spread.
    • Choroid plexus carcinomas (CPCs) grow more aggressively and are more likely to spread than either APPs or CPPs. CPCs are less common, accounting for 25-35 percent of all choroid plexus tumors.

    Choroid plexus papillomas are often easier to treat than carcinomas. An APP can act more aggressive but usually behaves like a CPP.

    What are the symptoms and causes of choroid plexus tumors?

    Brain tumors can cause a variety of symptoms in children depending on their size and location. Keep in mind that the symptoms of a brain tumor may resemble other more common conditions or medical problems. It is important to consult your child's physician for a diagnosis.

    Choroid plexus tumors symptoms are most often associated with increased pressure in the brain and can 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 – blockage of the normal flow of cerebrospinal fluid; pressure can increase in the brain and the skull can enlarge
    • Nausea and vomiting. These symptoms are 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

    As a parent, you undoubtedly want to know what may have caused your child’s tumor. It’s important to understand that these and other brain tumors most often occur with no known cause. There’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

    One rare genetic disease called Li-Fraumeni syndrome can be associated with CPC. If your child has a CPC, his or her doctors will talk to you about genetic testing for Li-Fraumeni syndrome.

    How are choroid plexus tumors diagnosed?

    The first step in treating your child is forming an accurate and complete diagnosis. Choroid plexus tumors are most commonly diagnosed from imaging studies and biopsy.

    Choroid plexus tumor diagnostic tests may include:

    • A physical exam and complete medical history
    • A neurological function test of reflexes, muscle strength, eye and mouth movement, coordination and alertness
    • Magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images of the structures within the brain and spine. An MRI uses a combination of large magnets and a computer to analyze organs and structures within the body. No x-rays or radiation are used.
    • Lumbar puncture (spinal tap), to remove a small sample of cerebrospinal fluid (CSF). This can determine if any tumor cells have spread. In children, this procedure can safely be performed under sedation.

    There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.

    What are the treatments for choroid plexus tumors?

    Your child's physician will determine a specific course of treatment based on several factors, including your child's age, medical history, the type, location, and size of the tumor and the extent of the disease.

    Choroid plexus tumor treatment may include:

    • Neurosurgery: For CPP, between 85 and 100 percent of children are treated successfully with complete removal of the tumor and need no further treatment. For patients with CPC, it’s important to remove as much of the tumor as possible but further treatment will be needed.
    • Chemotherapy: Chemotherapy are medications that interfere with the cancer cells' ability to grow or reproduce, and may be used before surgery, to shrink the tumor, or after surgery, to kill any remaining cancer cells. Different groups of chemotherapy drugs work in different ways and are generally systemic treatments. Your child may receive chemotherapy 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; or intrathecally, as a direct injection into the spinal fluid.
    • Radiation: High-energy waves from a specialized machine damage or shrink tumors, and are precisely targeted and dosed to kill cancer cells left behind after your child’s surgery. This treatment is important to control the local growth of tumor. Due to the potential side effects of radiation, including effects on learning and hormone function, it is best avoided if your child is young (especially under the age of 3 years).

    How are side effects of choroid plexus tumor treatment managed?

    Side effects in the treatment of choroid plexus tumors can arise from surgery, radiation therapy and chemotherapy. Knowing what these side effects are can help you and your child prepare for, and, in some cases, prevent these symptoms.

    • Procedures should be performed in specialized centers where experienced neurosurgeons, working in the most technologically advanced settings, with doctors who have the expertise to remove as much tumor as is safely possible while preserving normal brain tissue.
    • Radiation therapy often produces inflammation, which can temporarily exacerbate symptoms and dysfunction. To control this, steroids, which are anti-inflammatory medications, are sometimes necessary.
    • Chemotherapy drugs cannot tell the difference between normal, healthy cells and cancer cells. Some of the chemotherapy agents are associated with fatigue, hair loss, nausea, vomiting and headache. These side effects can be effectively managed under most circumstances.

    Our Brain Tumor Center also has access to specialists who deliver complementary or alternative medicines. These treatments, which may help control pain and side effects of therapy, include the following.

    • acupuncture/acupressure
    • therapeutic touch
    • massage
    • dietary recommendations

    Talk to your child’s physician about whether complementary or alternative medicines are a viable option.

    What is the latest research on choroid plexus tumors?

    Clinical Trials

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

    It’s possible that your child will be eligible to participate in one of our brain tumor clinical trials. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Neuro-oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group.

    If your child has progressive or recurrent tumor, she may be eligible for a number of experimental therapies

    Participation in any clinical trial is completely voluntary. We will take care to fully explain all elements of the treatment plan prior to the start of the trial, and you may remove your child from the medical study at any time.

    What is the long-term outlook for choroid plexus tumors?

    Patients with choroid plexus papilloma do extremely well with surgery. Patients with choroid plexus carcinoma 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. CPC requires additional postoperative treatment, with a survival rate of about 60-65 percent.

    There are a number of 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.

    Children who have completed treatment for a choroid plexus tumor should visit a survivorship clinic yearly to manage disease complications, screen for recurrence and manage late treatment side effects. A typical follow-up visit is likely to include a physical exam, laboratory testing and imaging scans.

    Through our Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic, children are able to meet with their pediatric neuro-oncologist and neurologists at the same follow-up visit. Our multidisciplinary approach and depth of expertise will give your child access to endocrinologists, neuro-psychologists and alternative/complementary therapy specialists. School liaison and psychosocial personnel from the pediatric brain tumor team are also available. In addition, children needing rehabilitation may meet with speech, physical, and occupational therapists during and after their visit.

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