Ependymomas are tumors that grow in the ependyma, the cells that line passageways in the brain where protective brain and spinal cord fluid (cerebrospinal fluid) is created.
The cause of ependymomas is not known, but they make up about six percent of brain tumors and about 60 percent of spinal cord tumors. While the average age of diagnosis is 22 years old, they also occur in children around age 5 and adults around age 34.
In adolescents and adults, many low-grade intracranial ependymomas, including those occurring in a patient’s spinal cord, can be completely removed surgically without long-term consequences. Higher-grade tumors tend to be resistant to treatment.
Diagnostic An Ependymoma
Imaging studies performed by a brain specialist (also known as neurologist) are the most effective way to locate and diagnose an ependymoma. They can include an MRI (Magnetic Resonance Imaging), CT Scan (Computed Tomography) and chest X-Ray. A biopsy – or piece or tissue removed from the tumor itself – may also be necessary to determine the type of tumor.
• MRI (Magnetic Resonance Imaging): A non-invasive scan that reveals detailed images of the brain and its surrounding nerves and tissue.
• CT Scan (Computed Tomography): While a surefire diagnosis comes from an MRI, a CT scan can also help identify the exact location and size of the tumor. A CT scan uses X-rays to make detailed images of structures inside the body.
• Chest X-Ray: A chest X-ray may be taken to see if the tumor has spread – or metastasized – from the brain to other parts of the body (or vice versa). However, X-rays are much less sensitive and are used less often to diagnose a brain tumor.
Brain tumors are categorized on four-level scale developed by the World Health Organization. Grade I tumors are the least aggressive, while Grade IV are the most aggressive.
• Grade I: These tumors have slow-growing cells that appear similar to normal brain cells.
• Grade II: Cells of Grade II tumors grow relatively slowly but show signs of change. They are typically considered “pre-cancerous,” as the tumor’s cells may begin to move into neighboring healthy brain tissue. If they are surgically removed, the tumor may return as a higher-grade.
• Grade III: The cells of Grade III tumors lack the structure and function of normal cells. They actively reproduce and can grow into neighboring healthy tissue. Again, if they are surgically removed it is not uncommon for the tumor to return as a higher-grade.
• Grade IV: Grade IV tumors are made up of the most aggressive, rapidly reproducing abnormal cells. These cells reproduce so quickly because they are able to form new blood vessels that support their aggressive growth.
Treatment & Risks
Each patient and ependymoma tumor is unique and requires a team of specialists that work together to develop a customized treatment plan. Depending on the location and size of the tumor, your personalized team may include one or more of the following specialists:
• Neuro-oncologist: A physician specializing in brain cancer.
• Neuropathologist: A physician specializing in diseases of the brain and spinal cord with the ability to make a definitive diagnosis of brain tumors.
• Neurosurgeon: A physician trained in surgery on the nervous system, especially the spine and the brain.
• Radiation oncologist: A physician specializing in the treatment of cancer patients, using radiation therapy as the main method of treatment.
Depending on the grade of the tumor, ependymomas are typically treated by three methods: observation, surgery and/or radiation. Because ependymomas can grow very slowly, lower-grade tumors may need nothing more than close observation. More aggressive ependymomas in the spine or brain will be removed surgically and any remaining tumor tissue may be treated with postoperative radiation therapy and/or chemotherapy. However, less than 20 percent of ependymomas respond to chemotherapy.
1. Observation: Because ependymomas can grow very slowly, lower-grade tumors may need nothing more than close observation. A physician will monitor any activity of the tumor through regular MRI scans. If the tumor shows signs of growth, it may be treated with surgery or radiation.
2. Surgery: The goal of surgery is to remove as much of the tumor as possible while minimizing damage to healthy brain tissue. The location and size of the tumor will determine how much of it, if any, can be removed. Removal of the tumor reduces the pressure within the brain, improving symptoms. If the entire tumor cannot be removed, the smaller, remaining area will be treated by radiation.
3. Radiation: In certain situations, complete surgical removal of the ependymoma may not be an option. For example, the neurosurgical team may need to leave part of the tumor in place to avoid damage to a vital structure and the possibility of serious or life-threatening consequences. In these cases radiation may be an appropriate treatment, either alone or following surgery. Cedars-Sinai’s treatment teams have several state-of-the-art focused radiation options. For ependymomas, the Gamma Knife® is an extremely precise, fixed system that uses up to 201 separate beams of radiation converging at a single point. The Trilogy™ System provides highly focused, image-guided radiotherapy and radiosurgery for treating ependymomas and other lesions that are in close proximity to the spinal cord or other vital structures. The goal of either approach is to bombard the tumor with a destructive dose of radiation while minimizing damage to healthy tissue.
A brain tumor can produce neurologic symptoms by putting pressure on important structures within the brain. As a tumor grows and takes up more space within the skull, it causes swelling inside the brain. Since the skull has a limited amount of space inside it, a tumor does not have to grow substantially to increase pressure and cause symptoms.
Most common symptoms include:
• Headaches, resembling pressure behind the eyes
• Vision loss, blurriness or double-vision
Less common symptoms include:
• Neck pain
• Personality changes, e.g. irritability
• Rapid and jerky eye movements