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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.
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):
Choroid plexus carcinomas (CPC):
CPP and CPC make up the vast majority of choroid plexus tumors. CPP are often easier to treat.
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.
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 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:
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.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”