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Choroid Plexus Brain Tumor

  • Choroid plexus brain tumors arise from the choroid plexus, tissue located in the spaces of the brain called ventricles. This tissue makes cerebrospinal fluid (CSF), the fluid that surrounds the brain and spinal cord.

    Having a tumor in the brain is always a very serious matter, and a choroid plexus tumor is no exception. However, most children and adolescents who have been diagnosed with one survive into adulthood. Many of them face physical, psychological, social and intellectual challenges related to their treatment, and these patients require ongoing care to help with school and with skills they will use throughout adulthood.

    As you read on, you’ll find detailed information about choroid plexus tumors. If you would like to read more general information about brain tumors first, see our overview on brain tumors.

    How Dana-Farber/Boston Children’s approaches choroid plexus tumor

    Children with choroid plexus brain tumors are treated through the Brain Tumor Center at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric oncology program through Dana-Farber Cancer Institute and Boston Children’s Hospital that provides—in one specialized program—all the services of both a leading cancer center and a pediatric hospital.

    After treatment, your child will receive expert pediatric brain tumor survivorship care through the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, where he will be able to meet with his neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologist at the same follow-up visit.

    The No. 1 predictor of how a child will do in an operation is not based on where the tumor is or how big it is. The No. 1 predictor is how experienced the neurosurgeon is in doing that operation in children.

    Mark Kieran, MD, PhD, director of the Brain Tumor Center

    Reviewed by Susan Chi, MD
    © Boston Children’s Hospital, 2013

  • What are choroid plexus tumors?

    These tumors arise from the choroid plexus, tissue located in the spaces of the brain called ventricles. This tissue makes cerebrospinal fluid (CSF), the fluid that surrounds the brain and spinal cord.

    These are rare tumors, representing only 3 percent of brain tumors in children and 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. They affect girls and boys equally.

    How are choroid plexus tumors classified?

    An important part of diagnosing a brain tumor involves staging and classifying the disease, which will help your child’s doctor determine treatment options and prognosis. Staging is the process of determining whether the tumor has spread and if so, how far.

    There are three main types of choroid plexus tumors: papillomas, atypical papillomas and carcinomas:

    Choroid plexus papillomas (CPP) and atypical papillomas (APP):

    • grow more slowly (benign)
    • are by far more common, accounting for 80 to 90 percent of choroid plexus tumors
    • only rarely spread to other parts of the brain and spinal cord

    Choroid plexus carcinomas (CPC):

    • grow more aggressively (malignant)
    • are more likely to spread (metastasize)
    • are less common, accounting for 10 to 20 percent of all choroid plexus tumors

    CPP and CPC make up the vast majority of choroid plexus tumors. CPP are often easier to treat.

    Can a choroid plexus papilloma turn into a choroid plexus carcinoma?

    In rare cases, a choroid plexus tumor may appear to have characteristics of both CPP and CPC. Generally speaking, these are two different kinds of tumors, and usually a CPP remains a CPP. Tumors have a wide spectrum of how dangerous they may be, and sometimes a CPP may develop some unusual features, but usually not enough to fulfill the criteria for a CPC. An APP may occasionally behave like a CPP.

    Causes of choroid plexus tumors

    What causes choroid plexus tumors?

    While one rare genetic disease called Li-Fraumeni syndrome can be associated with CPP, these tumors most often have no known cause. There’s nothing that you could have done or avoided doing that would have prevented this tumor from developing.

    If your child has a CPP, his doctors will talk to you about genetic testing for Li-Fraumeni syndrome.

    Symptoms of choroid plexus tumors

    What are the symptoms of a choroid plexus tumor?

    Symptoms vary depending on size and location of tumor. A choroid plexus tumor can block the normal flow of cerebrospinal fluid, causing increased pressure on the brain (hydrocephalus) and enlargement of the skull. It also can cause symptoms including:

    • headaches (generally upon waking in the morning)
    • nausea and vomiting (often worse in the morning and improving throughout the day)
    • lethargy and irritability
    • problems feeding or walking
    • enlarged fontanelles (the “soft spots" between the skull bones in infants)

    These symptoms may resemble other, more common conditions or medical problems. If you don’t have a diagnosis and are concerned, always consult your child's physician.

  • The first step in treating your child is forming an accurate and complete diagnosis, so your child’s physician may order a number of different tests. In addition to a medical history and physical and neurological exam (which tests reflexes, muscle strength, eye and mouth movements, coordination and alertness), your child’s doctor may request tests including:

    • magnetic resonance imaging (MRI) – a diagnostic imaging procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of the brain and spine.
    • lumbar puncture (spinal tap) – To remove a small sample of cerebrospinal fluid (CSF), a special needle is placed into the lower back, into the spinal canal, the area around the spinal cord. CSF is the fluid that surrounds the brain and spinal cord. The sample is sent for testing to determine if any tumor cells have started to spread. For children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter. 

    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 the best treatment options for choroid plexus tumors.

  • Children with choroid plexus brain tumors are treated through the Brain Tumor Center at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric oncology program through Dana-Farber Cancer Institute and Boston Children’s Hospital that provides—in one specialized program—all the services of both a leading cancer center and a pediatric hospital. Our Brain Tumor Center is a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors.

    Your child’s physician will determine a specific course of treatment for choroid plexus tumors based on several factors, including:

    • your child’s age, overall health and medical history
    • type, location and size of the tumor
    • extent of the disease
    • your child's tolerance for specific medications, procedures or therapies

    There are a number of treatments we may recommend. Some of them help to treat the tumor while others are intended to address complications of the disease or side effects of the treatment.

    Surgery for choroid plexus tumors

    Between 85 and 100 percent of children with CPP are treated successfully with complete removal of the tumor through pediatric neurosurgery. For patients with CPC, it’s important to remove as much of the tumor as possible, but additional treatment, usually radiation and chemotherapy, may be needed. Chemotherapy before surgery may help shrink the tumor, making it easier to remove.

    Chemotherapy for choroid plexus tumors

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.

    How is chemotherapy given?

    Your child may receive chemotherapy:

    • orally, as a pill to swallow
    • intramuscularly (IM), as an injection into the muscle or fat tissue
    • intravenously (IV), directly to the bloodstream
    • intrathecally (IT), with a needle directly into the fluid surrounding the spine

    Radiation therapy

    Our doctors also may use precisely targeted and dosed radiation therapy 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 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, can provide the most extensive resections (surgical removals) while preserving normal brain tissue.
    • Radiation therapy often produces inflammation, which can temporarily exacerbate symptoms and dysfunction. To control this, steroids (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, diarrhea, constipation and headache. These side effects can be effectively managed under most circumstances.

    Our Pediatric 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
    • herbs
    • dietary recommendations

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

    What is the long-term outlook for a child with a choroid plexus tumor?

    A child’s long-term health after treatment for a choroid plexus tumor varies significantly depending on the pathologic diagnosis, whether the tumor has spread and whether it could be completely removed through surgery. CPP are generally more successfully treated than CPC. Your doctor will discuss treatment options with you and your family, including clinical trials and supportive care.

    Today, the majority of children and adolescents diagnosed with pediatric brain tumors will survive into adulthood. However, many will face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

    What about progressive or recurrent disease?

    There are many standard and experimental treatment options for children with progressive or recurrent choroid plexus tumors.

    Resources and support

    There are also a number of patient and family support services at Dana-Farber/Boston Children's Cancer and Blood Disorders Center to help you and your family through this difficult time.

    When appropriate, our Pediatric Advanced Care Team (PACT) offers supportive treatments intended to optimize the quality of life and promote healing and comfort for children with life-threatening illness. PACT also can provide psychosocial support and help arrange end-of-life care when necessary.

    Long-term follow-up

    To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children's Cancer and Blood Disorders Center provides pediatric brain tumor survivorship support and care through the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic.

    More than 1,000 pediatric brain tumor survivors of all ages are followed by the Outcomes Clinic, a multidisciplinary program designed to address long-term health and social issues for families and survivors of childhood brain tumors. Some of the post-treatment services provided by the Outcomes Clinic include:

    • MRI scans to monitor for tumor recurrences
    • intellectual function evaluation
    • endocrine evaluation and treatment
    • neurologic assessment
    • psychosocial care
    • hearing, vision monitoring
    • ovarian dysfunction evaluation and treatment
    • motor function evaluation and physical therapy
    • complementary medicine

    As a result of treatment, children may experience changes in intellectual and motor function. Among several programs addressing these needs are the School Liaison and Back to School programs, which provide individualized services to ease children's return to school and maximize their ability to learn.

    In addition to providing thorough and compassionate care, our Outcomes Clinic specialists conduct innovative survivorship research and provide continuing education for staff, patients and families.

  • Dana-Farber/Boston Children's Cancer and Blood Disorders Center is one of nine institutions in the nation belonging to the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients.

    We are also home to the world’s largest pediatric low-grade astrocytoma research program and the Department of Defense’s Neurofibromatosis Clinical Trial Consortium.

    Clinical trials

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

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