Ependymoma
Ependymomas are a type of a tumor that form in the brain or spinal cord as a result of abnormal growth of ependymal cells.
What Is Ependymoma?
Ependymomas are a type of a tumor that form in the brain or spinal cord (central nervous system) as a result of abnormal growth of ependymal cells. Ependymal cells are found in the ventricular system [the fluid-filled spaces in the brain (ventricles) and the spinal cord (central canal)]. There are about 200 new cases of ependymoma tumors in children in the United States each year.
There are three different grades of ependymoma tumors, classified based on how aggressive the tumor cells appear under the microscope and how fast they grow (grades 1-3, with grade 3 being the most aggressive):
- Grade 1: Subependymoma or myxopapillary ependymoma
- Grade 2: Ependymoma
- Grade 3: Anaplastic ependymoma
What Causes Ependymoma?
There is no known cause for ependymomas. There is no evidence that any environmental factors cause this type of tumor, and it is usually not a condition that is passed down to offspring. Researchers believe that something is absent during normal development and growth of ependymal cells. Specifically, cells of the brain and spinal cord grow and divide as the body develops. During this cell division process, cells need to replicate their genetic material. Errors can take place during this process, leading to mutations (changes in the gene structure) which may allow ependymal cells to grow into tumors. These errors generally occur randomly and cannot be prevented.
What Are the Signs & Symptoms of Ependymoma?
Your child’s symptoms may vary depending on the size of the tumor and whether the tumor has grown or spread to other areas. The most frequently seen symptoms associated with ependymoma tumors include:
- Nausea
- Vomiting
- Headache
- Difficulty walking
- Numbness or weakness in the limbs or midsection
- Back pain
- Blurred vision
- Lethargy (lack of energy)
- Irritability
How Is Ependymoma Diagnosed?
Your child's healthcare provider will ask about your child's health history and symptoms. The provider will do a physical exam as well as neurological exam that will test reflexes, muscle strength, eye and mouth movement and coordination. Your child's healthcare provider may refer your child to a cancer specialist (oncologist). Your child may need tests such as:
- CT Scan - A CT scan uses a series of X-rays and a computer to take pictures of the head or body. CT scans are very quick imaging, usually no more than a few minutes of scanning (though preparation may take longer).
- MRI - An MRI uses large magnets, radio waves and a computer to make detailed pictures of the body. Contrast dye may be injected into your child's vein so that the tumor will be more visible. MRIs are very detailed imaging (provide much more information about the tumor), so take longer to perform (often 1-2 hours).
- Biopsy - Tumor cells are removed during surgery and sent to a lab for testing. This is done to find out the type of tumor (to finalize the tumor diagnosis) to determine how best to treat it and to understand how aggressive it is.
- Lumbar Puncture (Spinal tap) - Spinal fluid is removed (using a small needle injected into the lower back while the patient is asleep) and sent to a lab for testing. This is done to evaluate for the presence of tumor cells that have spread to the spinal fluid.
How Is Ependymoma Treated?
The best treatment for ependymoma is to surgically remove as much of the tumor as possible. Treatment then will be individualized based on your child’s age, the location of the tumor, the grade (how aggressive the tumor appears under the microscope), and whether the tumor has spread (metastasized). Radiation following surgery is common for many ependymomas, with consideration of chemotherapy, but some patients don’t need additional treatment after surgery. Your child’s treatment may include one or more of the following:
- Radiation Therapy - These are high-energy X-rays or other types of radiation. They are used to kill cancer cells or stop them from growing.
- Chemotherapy - These are medicines that kill cancer cells. One or more medicines may be given. Medicines may be oral or given through an IV or central line.
- Clinical Trials - A clinical trial is a way to test new treatments for cancer. Ask your child's healthcare provider if there are any treatments being tested that may work well for your child. Many new treatments are only available in clinical trials.
Your child may have short- or long-term problems from the tumor or from treatment. They may include:
- Damage to the brain or nervous system that cause problems with coordination, muscle strength, speech or eyesight
- Problems after surgery, such as infection, bleeding and problems with general anesthesia
- Infection and an increased risk of bleeding from chemotherapy
- Delayed growth and development
- Learning problems
- Problems with reproduction (infertility)
- Return of the cancer (recurrence)
- Increased risk for other cancers later in life
Speak with your child’s doctor about what you should watch for and what can be done to help prevent complications.
Survivorship – Outcomes and Life After Ependymoma Treatment
Improving the quality of life of survivors of brain and spine tumors is an area of much ongoing research and continued progress. Some patients have little to no long-term problems from their tumor or treatment, whereas other patients experience more significant side effects. All patients will continue to be followed by the oncology team after finishing treatment to ensure they are doing well and to offer appropriate resources, therapies, and subspecialty team services as needed. Patients will also continue to get surveillance imaging (MRIs) to ensure their tumor does not grow back; as the time from end of treatment increases, these scans will be spaced further apart.
Why Choose Nationwide Children’s Hospital?
The Neuro-Oncology Program at Nationwide Children’s Hospital offers clinical excellence in treating children, adolescents, and young adults with brain and spine tumors. Patients and their families will be supported by a multi-disciplinary team of providers, all dedicated to ensuring that the patient has the best possible outcomes. Depending on the specific tumor diagnosis and treatment plan, the patient and their family will be cared for by oncologists, neurosurgeons, radiation oncologists, clinical psychologists, rehabilitation medicine doctors, neurologists, endocrinologists, ophthalmologists, therapists (physical, occupational, speech, art, music), social workers, dieticians, and/or pharmacists. These team-members all work closely together with one another and the family to provide the best care for the patient.
Cancer Clinical Research
Nationwide Children’s Hospital is also a national leader in oncology research and clinical trials. There are many ongoing research studies aimed at improving outcomes for children and young adults with brain/spine tumors—understanding why these tumors develop and how to appropriately target them. We are members of all major pediatric brain tumor clinical trial consortia including Pediatric Brain Tumor Consortium (PBTC), Collaborative Network of Neuro-oncology Clinical Trials (CONNECT), Pacific Neuro-oncology Consortium (PNOC) and the Children’s Oncology Group (COG). We have many open clinical trials testing new drugs in difficult-to-treat tumors.
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