Choroid Plexus Tumor
Having a tumor in the brain is always a very serious matter, and a choroid plexus tumor is no exception. Choroid plexus tumors arise in the choroid plexus, tissue located in the spaces of the brain called ventricles.
While all brain tumors are life-threatening, 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 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.
- The choroid plexus makes cerebrospinal fluid (CSF), the liquid that surrounds the brain and spinal cord.
- Choroid plexus tumors are rare, representing only 3 percent of brain tumors in children
- They’re 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
- Girls and boys are equally affected
- The vast majority of choroid plexus tumors are either choroid plexus papillomas (CPP) or choroid plexus carcinomas (CPC). CPP are often easier to treat.
How Dana-Farber/Boston Children's Cancer and Blood Disorders Center approaches 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.
After treatment, your child will receive expert follow-up care through the Stop & Shop Neuro-Oncology Outcomes Clinic at Dana-Farber/Boston Children's, where he will be are able to meet with his neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologist at the same follow-up visit.
- Our pediatric brain tumor survivorship clinic is held weekly.
- In addition to meeting with your pediatric neuro-oncologists, neurologist and neurosurgeon, your child may also see one of our endocrinologists or alternative/complementary therapy specialists.
- School liaisons and psychosocial personnel from the pediatric brain tumor team are also available.
- If your child needs rehabilitation, he may also meet with speech, physical and occupational therapists during and after treatments.
We understand that you may have a lot of questions when your child is diagnosed with a choroid plexus tumor:
- What exactly is it?
- What are potential complications in my child’s case?
- What are the treatments?
- What are possible side effects from treatment?
- How will it affect my child long term?
We’ve tried to provide some answers to those questions here, and when you meet with our experts, we can explain your child’s condition and treatment options fully.
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.
In rare cases, a choroid plexus tumor may appear to have characteristics of both CPP and CPC.
Can a choroid plexus papilloma turn into a choroid plexus carcinoma?
Generally speaking, these are two different kinds of tumors, and usually a CPP stays a CPP. Tumors have a wide spectrum in terms of how harmful they are, and sometimes a CPP may develop some unusual features, but usually not enough to fulfill the criteria for a CPC. APP may occasionally behave like a CPP.
What causes choroid plexus tumors?
It’s important to understand that these tumors most often have no known cause. There’s nothing that you could have done or avoided doing that would have prevented them from developing. There is one rare genetic disease called Li-Fraumeni Syndrome that can be associated with CPP. If your child has a CPP, his doctors will talk to you about genetic testing for this disease.
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 can also cause symptoms including:
- headaches (generally upon awakening in the morning)\
- nausea and vomiting (often worse in the morning and improving throughout the day)
- lethargy and irritability
- problems feeding or walking
- enlarged fontanels (the soft "spot" that occurs before the bones in the head become solid) in infants
Keep in mind that 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.
Questions to ask your child’s doctor
After your child is diagnosed with a brain tumor, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.
Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.
If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too.
Some of the questions you may want to ask include:
- Has my child’s brain tumor spread?
- Can the tumor be treated with surgery?
- How long will my child need to be in the hospital?
- What are the possible short and long-term complications of treatment? How will they be addressed?
- What is the likelihood of cure?
- What services are available to help my child and my family cope?
Q: Will my child be OK?
A: A child’s long-term health after treatment for a choroid plexus tumor varies significantly depending on the whether the tumor has spread and whether it can be completely removed through surgery. Choroid plexus papillomas are generally more likely to be successfully treated than choroid plexus carcinomas. Your doctor will discuss treatment options with you and your family including clinical trials and supportive care.
Q: What is the expected outcome (prognosis) for children after treatment for choroid plexus tumors?
A: Choroid plexus papillomas and atypical choroid plexus papillomas are usually treated by surgical removal of the tumor. Choroid plexus carcinomas are often harder to treat. It’s important to remove the tumor, but treatment also requires additional therapies such as chemotherapy and/or radiation.
Q: What services are available to help my child and my family cope?
A: We offer a variety of services to help you, your child and your family get through this difficult time.
Q: Where will my child be treated?
A: Children treated on an outpatient basis through Dana-Farber/Boston Children's are cared for at the Jimmy Fund Clinic on the third floor of Dana-Farber Cancer Institute. If your child needs to be admitted to the hospital, she will stay at Boston Children's Hospital on the ninth floor of the Berthiaume Building.
Q: What kind of supportive or palliative care is available for my child?
A: 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 can also provide psychosocial support and help arrange end-of-life care when necessary.
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 to best diagnose the tumor. In addition to a physical exam, a medical history and neurological exam (which tests reflexes, muscle strength, eye and mouth movement, coordination and alertness), your child’s doctor may request tests including:
- computerized tomography scan (also called a CT or CAT scan) — a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images of the brain. CT scans are more detailed than general x-rays. If a choroid plexus tumor is suspected, your child may have a CT scan of the brain
- magnetic resonance imaging (MRI) — a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of the brain and spine.
- biopsy — in many cases, a tissue sample from the tumor will be taken through a needle during a simple surgical procedure to confirm the diagnosis
- 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 bathes 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 outline the best treatment options.
Treatment and care
We know how difficult a diagnosis of a brain tumor can be, both for your child and for your whole family. That’s why our physicians are focused on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We’ll work with you to create a care plan that’s best for your child.
Your child’s physician will determine a specific course of treatment 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
- how your child's doctors expects the disease to progress
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. These treatments include:
Between 85 and 100 percent of children with choroid plexus papilloma (CPP) are treated successfully with complete removal of the tumor through neurosurgery. For choroid plexus tumors, chemotherapy before surgery may help shrink the tumor, making it easier to remove. For patients with choroid plexus carcinoma (CPC), it’s important to remove as much of the tumor as possible, but additional treatment, usually radiation and chemotherapy, is needed.
Chemotherapy are drug treatments that work by interfering with the cancer cell's ability to grow or reproduce. 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, as an injection into the muscle or fat tissue
- intravenously, directly to the bloodstream (intravenously, or “IV”)
- intrathecally, directly into the spinal fluid with a needle (intrathecally)
Does chemotherapy cause side effects?
While chemotherapy can be quite effective in treating certain cancers, the drugs do not differentiate normal healthy cells from cancer cells. Because of this, your child may experience adverse side effects during treatment. Being able to anticipate these side effects can help you, your child and your care team prepare for, and, in some cases, prevent these symptoms from occurring.
What are common side effects and how are side effects managed?
Common side effects to chemotherapy given for treatment of choroid plexus carcinoma include fatigue, headache, diarrhea and constipation, weakened immune system and need for transfusions. These side effects can usually be effectively managed with standard medical approaches.
Our Pediatric Brain Tumor Program 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.
- therapeutic touch
- dietary recommendations
Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.
Our doctors may also use precisely targeted and dosed radiation to kill cancer cells left behind after your child’s surgery. This treatment is important to control the local growth of tumor.
We usually don’t use radiation therapy unless your child’s tumor has regrown after chemotherapy. 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 age 10).
What is the long-term outlook for a child with a choroid plexus tumor?
Today, the majority of children and adolescents diagnosed with pediatric brain tumors will survive into adulthood. However, many of them 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.
Dana-Farber Cancer Institute is one of nine institutes in the nation belonging to the Pediatric Oncology Experimental Therapeutic Investigators Consortium. The consortium is dedicated to the development of new and innovative treatments for children with newly diagnosed as well as progressive or recurrent choroid plexus tumors and other brain tumors. We are also home to the world’s largest pediatric low-grade astrocytoma research program and the Department of Defense Neurofibromatosis Clinical Trial Consortium.
To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children's established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic.
Today, more than 1,000 pediatric brain tumor survivors of all ages are followed by the Outcomes Clinic, a multi-disciplinary 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.
Research and Innovations
Dana-Farber/Boston Children's is a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed (returning) or refractory (resistant to treatment) disease. It is also the New England Phase I Center of the Children's Oncology Group and a member of the Department of Defense Neurofibromatosis Clinical Trial Consortium.
We are home to the only dedicated pediatric low-grade glioma program, the Pediatric Low-Grade Astrocytoma (PLGA) Research Program.
For many children with rare or hard-to-treat conditions, clinical trials provide new options.