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Subtypes of choroid plexus tumors include: atypical choroid plexus papilloma, choroid plexus carcinoma, choroid plexus papilloma
Choroid plexus tumors are rare tumors that start in the brain’s ventricles. Ventricles are spaces in the brain that fill with cerebrospinal fluid.
Choroid plexus tumors can be either benign or malignant.
Every year 75 to 80 children and young adults under 21 in the U.S. are diagnosed with a choroid plexus tumor. They are most common in infants. But people can develop choroid plexus tumors at any age.
There are 2 main types of choroid plexus tumor:
Most choroid plexus carcinomas occur in infants and children younger than 5. Increased risk for choroid plexus carcinoma is associated with Li-Fraumeni syndrome, an inherited syndrome.
With complete tumor removal, choroid plexus papilloma (CPP) survival rates are nearly 100%. Choroid plexus carcinoma (CPC) is more aggressive. But the chance of cure is about 50-70%.
Factors that influence prognosis include:
The main treatment for a choroid plexus tumor is surgery.
In some cases, doctors recommend more treatments like chemotherapy and radiation therapy. Treatment needs vary depending on:
Symptoms vary based on the child’s age and tumor location.
As the tumor grows, cerebrospinal fluid is blocked and can build up in the brain. This is called hydrocephalus. The fluid causes the ventricles to widen. It increases pressure on the brain (intracranial pressure).
Increased pressure against brain tissue causes many of the symptoms.
Choroid plexus tumor symptoms may include:
Doctors test for choroid plexus tumors in several ways. These tests include:
Choroid plexus tumors may be benign or malignant. Tumors can be grouped by how they look under the microscope. Staging involves an MRI of the spine and brain and a lumbar puncture. The more abnormal cells look, the higher the grade.
Here is how they can be grouped:
Treatment for choroid plexus tumors may include surgery, chemotherapy, and radiation.
Choroid plexus papillomas and atypical choroid plexuses tumors are managed with surgery when possible.
Choroid plexus carcinoma is a more aggressive cancer. Most of these patients will receive many types of treatments.
Treatment depends on several factors:
Children who are long-term survivors of CPT need close and prolonged monitoring for late effects of the treatment.
Children with a germline TP53 mutation or Li-Fraumeni syndrome need a referral for genetic counseling. They also need surveillance for other associated cancers.
Reviewed: October 2021