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Learn MoreSubtypes 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:
Choroid plexus tumors begin in the ventricles of the brain. Ventricles are spaces in the brain that are filled with cerebrospinal fluid.
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:
Surgery is used to both diagnose and treat choroid plexus tumors. The goal of surgery is to remove as much of the tumor as possible. Rarely, surgery may not be possible because of where the tumor is.
Children with choroid plexus papilloma may not need more treatment if the tumor is removed.
Children with choroid plexus carcinoma usually need chemotherapy and radiation along with surgery.
In some cases, a shunt is placed in the brain to keep fluid from building up (hydrocephalus). This fluid causes increased intracranial pressure. It may cause many of the symptoms associated with choroid plexus tumors.
A shunt is a small tube that drains cerebrospinal fluid to remove it from the brain. It can lessen symptoms. The shunt may be temporary or permanent.
A shunt is a small tube that drains cerebrospinal fluid to keep it from building up in the brain. This fluid causes increased pressure inside the brain and may cause other symptoms of choroid plexus tumors.
Radiation therapy is sometimes used in addition to other treatments. The type of radiation therapy used depends on the location of the tumor and if it has spread. The child’s age is an important consideration in the use of radiation, too.
Radiation therapy uses beams of radiation, X-rays or protons, to shrink tumors and kill cancer cells. Radiation works by damaging the DNA inside cancer cells.
Supportive care for patients with choroid plexus tumors includes rehabilitation and neurological care. Steroid and anti-seizure medications may be needed.
More support can address issues in learning, developmental milestones, and coping with cancer.
A shunt is a small tube that drains cerebrospinal fluid to keep it from building up in the brain. This fluid causes increased pressure inside the brain and may cause other symptoms of choroid plexus tumors.
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.
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Reviewed: October 2021
