Choroid plexus tumor (CPT)
A choroid plexus tumor is a tumor that develops in the cells lining the inside surface of the brain that produce cerebrospinal fluid. Each year, about five children in the Netherlands are diagnosed with a choroid plexus tumor. The tumor can occur in children of all ages. In some cases, the tumor can spread to other parts of the central nervous system.
Different types
There are three types of choroid plexus tumors. They differ in how the tumor behaves and which treatment is needed.
Choroid plexus papilloma type A
This tumor does not spread. If it can be completely removed during surgery, it will not come back. This is the most common type. We see this tumor more often in younger children.Choroid plexus papilloma type B
This tumor can come back after surgery or spread. It can usually be treated well with chemotherapy and sometimes radiation therapy. The treatment depends in part on your child’s age.Choroid plexus carcinoma
This is a rare and more aggressive tumor. At the time of diagnosis, it may already have spread to the brain or spinal cord. Surgery is always needed. This is followed by additional treatment with chemotherapy and radiation therapy. We prefer not to use radiation therapy in young children under three to four years of age. This tumor is less common and occurs more often in older children.
Causes
A choroid plexus tumor develops during normal brain development. During cell division, changes occur in the DNA of the cell. This leads to the formation of a tumor.
Some children have an inherited predisposition to developing tumors. In most children, the tumor develops by chance during normal brain development.
Symptoms
The symptoms depend on the location of the tumor. The brain is surrounded by cerebrospinal fluid, which protects it. The body produces new cerebrospinal fluid every day and absorbs it into the bloodstream.
There is a high chance that the tumor blocks the flow of cerebrospinal fluid. The fluid cannot drain properly, which increases pressure inside the head. Your child may develop headaches. Nausea and vomiting can also occur.
How do we make the diagnosis?
An MRI scan of the head and spinal cord is performed to see where the tumor is located. A sample of cerebrospinal fluid is also taken.
Treatment
Children with a CPT are treated according to a protocol developed by national and international experts. In the case of a choroid plexus papilloma, surgical removal of the tumor by the neurosurgeon is almost always sufficient.
If additional treatment is needed, it consists of chemotherapy and, if the child is older than four years, radiation therapy.
Chance of cure
For a choroid plexus papilloma, the chance of cure is very high. Almost all children are cured of this tumor.
For a choroid plexus carcinoma, the chance of cure is lower. Sometimes the tumor comes back. If that happens, we cannot cure the disease at this time. Treatment then focuses on slowing the disease and maintaining quality of life.
Every child and every situation is unique. That is why we discuss each treatment plan in a large multidisciplinary team, called a tumor board. Together, we decide what is best for your child at that moment. We work closely with other hospitals in Europe and beyond. This allows us to share knowledge and experience. We follow the latest developments and actively contribute to research. In this way, we aim for the best possible cure and quality of life for your child.
Questions?
Children and adolescents with a choroid plexus tumor are treated in the neuro-oncology department.