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.
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.
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.
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.
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.
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).
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.
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 Pediatric Neuro-Oncology Consortium (PNOC). We are also the New England
Phase I Center of the Children's Oncology Group.
If your child has a 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.
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.