Ependymoma is a rare type of tumor that starts in the brain or spinal cord. It can occur in both children and adults. In adults, it's most often in the spinal cord.
An ependymoma may not be cancer (benign), or it may be cancer (malignant). A malignant ependymoma can spread, a benign one cannot. Both can cause problems as they grow and press on nearby tissues.
How does an ependymoma grow?
Your brain and your spinal cord make up your central nervous system (CNS). Ependymoma is a primary CNS tumor. This means it starts in the tissues of the brain or spinal cord instead of starting in another part of the body and spreading to the brain. Any tumor in the brain can cause problems by pressing against the brain and causing symptoms.
Ependymoma is a kind of glioma. This tumor starts in support cells of the brain called glial cells. The tumor is caused by out-of-control growth of a type of glial cell called ependymal cells. These cells line fluid-filled chambers of the brain called ventricles. The ventricles contain the cerebrospinal fluid (CSF). This is the fluid that surrounds and protects the brain and spinal cord. The cancer cells can spread in the CSF to other places in the brain or spinal cord. These tumors don’t often spread beyond the central nervous system.
Ependymomas are grouped by size and shape of the cells, how fast they grow, and where they are, as well as tumor size, consistency, and spread.
There are 5 types of ependymoma tumors:
Subependymoma (Grade I). This is a benign tumor of the ventricles. It usually grows slowly.
Myxopapillary ependymoma (Grade I). This is a slow growing spinal cord tumor.
Classic ependymoma (Grade II). This type is most common. It grows a bit faster.
RELA fusion-positive ependymoma (Grade II or III with a change in the RELA gene)
Anaplastic ependymoma (Grade III). This is a tumor often near the base of the brain. It grows faster than the other types.
What causes an ependymoma?
Researchers don't yet know what causes these tumors. The risk factors for these kinds of tumors aren’t known. There's no known way to prevent them.
What are the symptoms of an ependymoma?
Symptoms can vary from person to person. The symptoms also depend on where the tumor is. For instance, a tumor near the base of the brain may block the normal flow of CSF. This can increase the pressure in your skull. This may cause headaches, nausea, vomiting, and dizziness.
Your symptoms may be sudden or they may start slowly and get worse over time. In general, signs and symptoms of ependymoma can include:
Nausea and vomiting
Pain that wakes you up
Dizziness and balance problems
Eye problems, such as double or blurry vision
Weakness or numbness in an arm or leg
Bowel or bladder problems
Back pain that spreads to the arms or legs
How is an ependymoma diagnosed?
Ependymoma can be hard to diagnose. It's a rare tumor in adults. And it may be hard to tell the difference from other types of tumors. Your healthcare provider may refer you to a neurologist. This is a doctor who specializes in diseases of the central nervous system. You may also see a neuro-oncologist. This is a doctor who specializes in cancers of the brain and spinal cord. Or, you may be referred to a neurosurgeon. This is a surgeon who does brain or spinal cord surgery.
The process starts with a medical history and a physical exam. Your healthcare provider will ask about your symptoms and medical history. You may also be asked about your family’s medical history.
The physical exam may include a neurologic exam. During this exam, your doctor may ask you to do things such as walk, touch your finger to your nose, or hold your hands out. He or she may tell you to follow a light with your eyes. You may also need tests such as:
MRI. This test uses magnets and a computer to create images of the inside of the body. MRI scans of your brain and spinal cord are used to get more information about the tumor. Contrast dye may be used to help show more detail in the images.
Lumbar puncture. This test is also called a spinal tap. A needle is used to take out a small amount of CSF from the space around your spine in your lower back. The fluid is checked for tumor cells.
A biopsy is usually needed to diagnose an ependymoma. Surgery will be done to take out a small piece of the tumor or try to remove the tumor. The removed tissue is tested in a lab to learn more about the tumor.
How is an ependymoma treated?
Your healthcare team will work with you to create a treatment plan. Your team may include:
Treatment depends on the type of tumor and where it is. The main treatment is surgery. This is done to remove as much of the tumor as possible. A doctor will test the tumor to find out the type, the size of the tumor, and learn more about it.
You may need radiation therapy or chemotherapy after surgery. Radiation therapy is done by sending beams of energy to the site of the cancer. Chemotherapy is done with medicines that kill cancer cells. They may be given by mouth, IV, or injected into your spinal fluid (intrathecal).
You will be watched closely after treatment. In some cases, an ependymoma comes back after treatment. Your provider will watch for this, as well as long-term treatment side effects. You'll likely need to have follow-up MRI scans every 3 or 4 months for the first year, and then a few times a year after that.