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Ependymoma is a rare type of tumor of the brain or spinal cord. It can happen in both children and adults. In children, these tumors tend to be in the brain. In adults, they are often in the spinal cord.
Your brain and your spinal cord make up your central nervous system (CNS). Ependymoma is a primary CNS tumor. This means that it starts in the brain or spinal cord rather than starting somewhere else in the body and spreading to the brain.
Ependymoma is a kind of glioma. This is a tumor that develops from support cells (glial cells) of the brain. These tumors arise from abnormal growth of a certain type of glial cells, the ependymal cells. These cells line the ventricles of the brain. The ventricles are the chambers that contain the cerebrospinal fluid (CSF) that bathes and cushions the brain and spinal cord. Ependymomas may spread within the CSF to other places in the brain or spinal cord. These tumors don’t typically spread beyond the CNS.
Most ependymomas are relatively slow-growing tumors. There are 4 types:
A tumor in the brain may cause problems by pressing against part of the brain and causing symptoms.
Researchers do not yet know what causes these tumors. Healthcare providers also do not have a clear idea about the risk factors for these tumors. Certain viruses may play a role in causing these tumors.
The symptoms depend on where the tumor is. For example, a tumor near the base of the brain may block the normal flow of CSF. This can lead to increased intracranial pressure (ICP). This may cause you to have a headache, nausea, vomiting, and dizziness. Your symptoms may be sudden or they may start slowly and gradually worsen. People with spinal cord tumors may have localized pain for months or even years before other symptoms develop. In general, signs and symptoms of ependymoma can include:
Your symptoms may vary.
Ependymoma can be difficult to diagnose because it is a rare tumor in adults. It may be hard to tell the difference from other types of tumors. Your primary healthcare provider may refer you to a neurologist. This is a healthcare provider who specializes in diagnosing and treating diseases of the central nervous system. You may see a neuro-oncologist. This is a healthcare provider who specializes in cancers of the brain and spinal cord. You may be referred to a neurosurgeon. This is a surgeon who performs 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 past medical conditions. He or she may also ask about your family’s medical history. The physical exam may include a neurologic exam. During a neurologic exam, your healthcare provider may ask you to do things like 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. Tests may be done, such as:
Your healthcare provider will put together a pathology report. This report tells the size, location, type, grade, and other specific information about your tumor.
You and your medical team will decide on the best treatment plan for you. These team members may include:
Treatment depends on the type and location of your tumor. Your healthcare team will decide the best treatment plan for you. The main treatment in adults is surgery to remove as much of the tumor as possible. Your chances of the best outcome are highest if your healthcare provider is able to fully remove your tumor. A healthcare provider will examine the tumor to find out your tumor type and grade.
Radiation therapy often follows surgery. In some cases, you may need chemotherapy after surgery. You may also have one or more of the following treatments:
You may also need a tube inserted to drain excess CSF and lower your ICP. You will likely need to have several follow-up MRI scans to monitor your situation.
Most people with ependymomas have a good outcome. In some cases, ependymoma comes back after treatment. To check for this, you will likely need to have follow-up MRI scans in the weeks after surgery, and then a few times a year after that. Talk to your healthcare provider about the risk of treatment risks and side effects, your prognosis, and the chances of your tumor returning.
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