Choroid Plexus Tumors Diagnosis and Treatment

Choroid plexus tumors are primary central nervous system (CNS) tumors. This means they begin in the brain or spinal cord.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of choroid plexus tumors?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Choroid plexus tumors are grouped in three grades based on their characteristics (grade 1, 2, or 3, also written as  grade I, II, or III) .

1. Grade 1 choroid plexus papilloma are low grade tumors. This means the tumor cells grow slowly.
2. Grade 2 atypical choroid plexus papilloma are mid-grade tumors. This means the tumors have a higher chance of coming back after being removed.
3. Grade 3 choroid plexus carcinoma are malignant (cancerous). This means they are fast-growing tumors that tend to invade nearby tissue.

What do choroid plexus tumors look like on an MRI?

Choroid plexus tumors usually appear in the ventricles of the brain. They usually have irregular borders and a “cauliflower-like” appearance. The tumors usually enhance with contrast and sometimes many tumors can be seen in a different region of the CNS. Choroid plexus carcinomas may also have some swelling around them, which can be seen during an MRI. Hydrocephalus is almost always seen, too.

What causes choroid plexus tumors?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. The cause of most choroid plexus tumors is not known. Genetic changes have been linked to the formation of some choroid plexus tumors. Rarely, certain gene changes that can be passed down through families have been linked to a higher chance of developing choroid plexus carcinomas.

Where do choroid plexus tumors form?

Choroid plexus tumors arise from a structure in the brain called the choroid plexus. It lines the ventricles (fluid-filled cavities) of the brain and its primary function is to produce cerebrospinal fluid (CSF). Choroid plexus tumors almost always form within the ventricles. They can also form in other regions of the CNS.

Do choroid plexus tumors spread?

Choroid plexus tumors can spread to other areas of the CNS through CSF.

What are the symptoms of a choroid plexus tumor?

Symptoms related to choroid plexus tumors depend on the tumor’s location. Here are some possible symptoms that can occur.

Choroid Plexus Tumors Symptoms

People with choroid plexus tumors may have increased pressure within the skull due to the production of too much CSF or blockage of its normal flow. This problem is known as hydrocephalus.

Signs and symptoms of hydrocephalus may include:

• Nausea
• Vomiting
• Irritability
• Headaches
• Blurred or double vision
• A strong desire to sleep
• Seizures

Who is diagnosed with choroid plexus tumors?

Choroid plexus tumors occur in both children and adults, but are more common in children in the first year of life. Choroid plexus tumors occur slightly more often in females than males. An estimated 1,423 people are living with this tumor in the United States.

What is the prognosis of choroid plexus tumors?

The likely outcome of the disease or chance of recovery is called prognosis.

Choroid Plexus Tumors Prognosis

The relative 5-year survival rate for choroid plexus tumors is 63.9% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are the treatment options for choroid plexus tumors?

The first treatment for choroid plexus tumors is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person.

Treatments after surgery may include radiation, chemotherapy, or clinical trials. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.

Open Clinical Studies for Choroid Plexus Tumors

• Outcomes and Risk Project for Patients with Rare CNS Cancers
• Evaluation of the Natural History and Specimen Banking for Patients with CNS Cancers
• Virtual Reality Study for Patients with Brain Cancer
• Sleep Observation Study for Patients with Brain Cancer
• CALM Therapy Intervention Study for Patients with Brain Cancer
• Immune Checkpoint Inhibitor Nivolumab for Patients with Rare CNS Cancers
• ONC206 for Patients with Rare CNS Neoplasms

Learn More

• Collaborating Globally to Impact Outcomes for Rare Brain and Spine Cancers
• Inspiring Messages from the Brain Tumor Community
• Advice from the Brain Tumor Community
• Living with a Brain or Spine Tumor