Choroid plexus tumors are rare tumors of the central nervous system (CNS), meaning they begin in the brain or spinal cord. They account for less than 1% of all brain tumors, but 10% to 20% of these tumors occur in babies under the age of 1. Increased pressure within the brain and hydrocephalus (buildup of fluid) can cause symptoms like headaches and confusion. Surgery is the first line of treatment.
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What Is A Choroid Plexus Tumor?
Choroid plexus tumors arise from a brain structure called choroid plexus, which lines the ventricles – the fluid-filled spaces in the brain. The primary function of the choroid plexus is to produce and circulate spinal fluid. Choroid plexus tumors can form within the ventricles as well as other regions of the central nervous system (CNS).
Choroid plexus tumors can cause symptoms similar to other intraventricular tumors, with headache and confusion as the most common symptoms. While adults can also get these tumors, they mostly affect young children.
In babies, these tumors usually appear in the upper part of the brain, especially on the left side of the lateral ventricles. In adults, they tend to show up in the lower part of the brain, often in the fourth ventricle.
What are the types of choroid plexus tumors?
Choroid plexus tumors are classified based on a neuropathologist’s analysis of the tumor tissue. They are divided into three grades —grade 1, 2, or 3, according to their specific features.
- Grade 1 choroid plexus tumors, known as choroid plexus papillomas, are benign (noncancerous) and tend to grow slowly.
- Grade 2 tumors, known as atypical choroid plexus papillomas, are also considered benign but are classified as intermediate-grade because they have a greater potential to recur in the same location after removal.
- Grade 3 tumors, also known as choroid plexus carcinomas, are malignant. These cancerous tumors grow quickly and are more likely to spread to other parts of the central nervous system.
While grade 1 and 2 tumors rarely metastasize within the CNS, as many as 20% of grade 3 tumors have already spread to multiple areas in the CNS by the time they are diagnosed.
How common are choroid plexus tumors?
Choroid plexus tumors are rare brain tumors that can occur in both children and adults, but are more common in children, especially during the first year of life. The average age at diagnosis is around 3.5 years, and about 70% of cases are found in kids under 2 years of age.
They make up less than 5% of all pediatric brain tumors but represent more than 10% of brain tumors diagnosed in babies during the first year of life. In children, they are the third most common type of brain tumor present at birth, after teratomas and gliomas.
In adults, these tumors are even rarer, accounting for less than 1% of all adult brain tumors diagnosed. About 2,340 people in the U.S. are living with this type of tumor.
What causes choroid plexus tumors?
The cause of many choroid plexus tumors is unknown.
In about half of cases, genetic mutations (changes) of the TP53 gene have been linked to the formation of grade 3 choroid plexus carcinomas. Rarely, certain inherited genetic mutations have been linked to a higher chance of developing choroid plexus carcinomas. Some choroid plexus tumors are linked to genetic conditions like Aicardi syndrome, hypomelanosis of Ito, chromosome 9p duplication, and von Hippel-Lindau syndrome.
Choroid plexus tumor risk factors
The people at the highest risk of choroid plexus tumors are those with Li Fraumeni syndrome (a germline or constitutional mutation in the TP53 gene), which puts them at much higher risk of developing choroid plexus carcinoma than the average population.
Complications of choroid plexus tumor
If choroid plexus tumors are left untreated, they can lead to serious complications, including a condition called hydrocephalus, which is an abnormal buildup of fluid in the brain. This can result in symptoms ranging from headaches and confusion to loss of vision and seizures.
Therefore, it’s crucial to seek medical attention if you suspect a choroid plexus tumor. Early diagnosis and treatment can help manage symptoms, prevent complications, and improve the overall prognosis.
What Are the Signs and Symptoms of A Choroid Plexus Tumor?
The main symptoms of choroid plexus tumors are:
- Confusion
- Headaches
Other common symptoms may include:
- Nausea or vomiting.
- Personality changes.
- Seizures.
- Speech, vision, or memory problems.
- Weakness or loss of sensation in the arms and/or legs.
When should I see a doctor about my choroid plexus tumor symptoms?
Because choroid plexus tumors can cause increased intracranial pressure and affect brain function, early evaluation and diagnosis are important for the best possible outcomes. Prompt medical assessment can lead to earlier intervention and better management of symptoms.
How Do You Diagnose A Choroid Plexus Tumor?
To help diagnose choroid plexus tumors, your doctor will ask you about your symptoms and order imaging tests.
Tests to diagnose a choroid plexus tumor
Your doctor can identify a choroid plexus tumor using imaging studies such as MRI or CT scans.
On imaging, choroid plexus tumors typically look cauliflower-like, with irregular borders and swelling in the brain’s ventricles. An abnormal buildup of fluid, called hydrocephalus, is also common.
Choroid plexus tumor prognosis
Many factors affect prognosis, including tumor grade, location, type, extent of spread, age, and health of the person, and the amount of tumor left after surgical resection (removal).
Generally, the relative five-year survival rate in U.S. adults age 20 and older is 71%. The five-year survival rates for choroid plexus by grade are:
- Grade 1 choroid plexus papilloma — Nearly 100%.
- Grade 2 atypical choroid plexus papilloma — Approximately 85%.
- Grade 3 choroid plexus carcinoma — Between 40% and 60%; less than 30% if TP53 mutation present.
How Do You Treat A Choroid Plexus Tumor?
Surgery is usually the primary treatment for choroid plexus tumors. The goal of surgery is to remove as much tumor as possible without affecting healthy tissue, and to get a tissue sample that can be used to determine the tumor type.
Surgery can usually cure choroid plexus papillomas, which are noncancerous.
People with choroid plexus carcinomas — the malignant (cancerous) type of choroid plexus tumor — usually require additional treatments, such as radiation therapy and chemotherapy.
Our neurosurgical team may recommend a combination of surgical and nonsurgical approaches for you to maximize the benefits of surgery while minimizing risks.
Minimally invasive surgery
At UPMC, the preferred surgical treatment for choroid plexus tumors is Neuroendoport® surgery.
Neuroendoport surgery offers a minimally invasive option for deep-seated tumors in brain tissue and tumors within the fluid-filled ventricles of the brain. A narrow tube or port allows surgeons to access these tumors through a tiny incision in the scalp, in contrast to traditional open brain surgery.
Neuroendoport surgery gives surgeons access to the tumor through a dime-size channel. This minimally invasive approach offers benefits such as:
- Faster recovery times than with traditional surgery.
- Fewer side effects and complications.
- Minimal scarring.
Radiation therapy
Radiation is a common treatment for brain tumors, either alone or in combination with surgery and/or chemotherapy.
Radiation therapy may be delivered in several ways:
- Externally, by directing radiation at the choroid plexus tumor from an outside source.
- Internally, by placing radioactive material directly in the area of the brain near the cancer.
- Using stereotactic radiosurgery (SRS), which sends a concentrated dose of radiation directly to the tumor.
Chemotherapy
Chemotherapy treatment uses drugs to stop the growth of cancer cells and may be used to treat choroid plexus carcinoma.
Depending on the stage of your cancer, you might receive chemotherapy by mouth, by injection, or directly into the site of the tumor.
