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
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
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 are often easier to treat than
carcinomas. An APP can act more aggressive but usually behaves like a CPP.
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:
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.
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:
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.
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:
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
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.
Talk to your child’s physician about whether complementary or alternative
medicines are a viable option.
For many children with brain tumors or other rare or hard-to-treat conditions, clinical trials provide new options.
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
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.
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
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
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
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