Choroid Plexus Tumors
Choroid plexus tumors are primary central nervous system (CNS) tumors. This means they begin in the brain or spinal cord.
What are the grades of choroid plexus tumors?
Choroid plexus tumors are grouped in three grades based on their characteristics.
- Grade I choroid plexus papilloma are low grade tumors. This means the tumor cells grow slowly.
- Grade II atypical choroid plexus papilloma are mid-grade tumors. This means the tumors have a higher chance of coming back after being removed.
- Grade III choroid plexus carcinoma are malignant (cancerous). This means they are fast-growing tumors that tend to invade nearby tissue.
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 males than females.
What causes choroid plexus tumors?
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. 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, and seizures.
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
- Immune Checkpoint Inhibitor Nivolumab for Patients with Rare CNS Cancers
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.
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