What is Ependymoma?
An ependymoma is a tumor that affects the brain or spinal cord. It is a primary central nervous system (CNS) tumor, meaning that it begins in the CNS and does not migrate from somewhere else in the body. The tumors originate in the ependymal cells that line the spinal column and spaces (ventricles) inside the brain.
Types of Ependymoma
Ependymomas are assigned grades that describe their growth pattern and other characteristics. The grade will influence the tumor’s prognosis and the appropriate course of treatment.
- Grade I (Subependymoma). This type of tumor develops near the ventricles inside the brain. This grade grows slowly and often doesn’t cause symptoms.
- Grade I (Myxopapillary ependymoma). This type of tumor affects the lower part of the spinal column. This grade also grows slowly and often doesn’t cause symptoms.
- Grade II (Classic). This type of tumor grows more quickly than a Grade I tumor and can occur in the brain or spine.
- l Grade III (Anaplastic). This type grows more quickly than Grade I or Grade II tumors. Grade III tumors are also likely to invade surrounding tissue and are more likely to recur.
Symptoms of Ependymoma
Slow-growing ependymomas may not produce symptoms or only mild symptoms that can go unnoticed. However, untreated tumors can grow large enough to produce severe symptoms. The specific symptoms experienced can vary depending on the location and size of the tumor.
Common symptoms of ependymomas include:
- Poor balance
- Trouble walking
- Vision difficulties
- Weakness or numbness in the arms or legs
- Back pain
- Bowel or bladder dysfunction
- Sexual dysfunction
What Causes Ependymoma?
The root cause of a brain tumor is a mutation or damage in the genes that control the growth of affected cells. The specific cause of the gene damage that triggers a tumor’s formation is usually not identifiable. In healthy cells, these genes prevent the cell from growing or reproducing too rapidly, and the genes can also determine the cell’s expected lifespan. In a tumor’s cells, the damage to the genes causes the cells to grow and reproduce rapidly, and the cells may live longer than usual. As this rapid growth and reproduction continue, the cells grow into an abnormal mass.
Is Ependymoma Hereditary?
Most ependymomas do not appear to be linked to inherited traits. Instead, researchers believe most gene changes that cause tumors come from external environmental factors or changes within cells that occur randomly and with no external trigger. However, some types of ependymoma have been associated with the inherited disorder neurofibromatosis type 2.
How Is Ependymoma Detected?
Slow-growing ependymomas may develop for years without producing symptoms, and some tumors never cause noticeable symptoms. Because their presence is subtle, ependymomas are sometimes discovered by chance when a patient undergoes an imaging scan for some other reason.
Some warning signs of ependymomas include:
- Nausea or vomiting
- Balance problems
How Is Ependymoma Diagnosed?
Doctors may take several different diagnostic steps when they suspect a patient may have an ependymoma.
- Neurological exam. A basic neurological exam will test a patient’s reflexes, balance, coordination, strength, vision, and hearing. This exam may prompt a doctor to look further for a tumor’s presence, giving a clue to the affected part of the brain.
- Imaging. Imaging technologies are non-invasive ways to look at brain tissue and possibly detect a tumor’s presence. They may also be used to judge the tumor’s size, location, and growth. Magnetic resonance imaging (MRI) uses a strong magnetic field to produce images of the brain and central nervous system. Computerized tomography (CT) scan may also be used to look for tumors.
- Biopsy. Doctors may require a biopsy, in which a sample of the tumor is removed and analyzed by a pathologist. The biopsy might be conducted with surgery or, if the tumor is in a particularly hard-to-reach area, using a needle guided by imaging technology. A pathologist’s examination of the tissue sample can help suggest the best treatment course.
How Is Ependymoma Treated?
Ependymoma treatment can vary depending on the tumor’s growth rate, location, and size.
The most direct way to treat a brain tumor is to remove as much of it as possible with surgical intervention. Typically, the surgery involves opening the skull and removing the tumor while not damaging the surrounding healthy tissue. In many cases, the borders of an ependymoma are well-defined, and the surgeon can remove all of the abnormal cells.
However, when a tumor is located in an especially sensitive area or has infiltrated surrounding brain tissue, the surgeon may not be able to remove all of the tumor, and other subsequent treatment options may be necessary.
Radiation therapies involve using high-energy x-rays to target and kill tumor cells directly. The radiation is typically focused on the tumor so that they do not damage healthy cells. Radiation therapy is often used when the tumor can’t be entirely removed with surgery or when the tumor is in a location that is not safely accessible.
Side effects of radiation therapy may include headaches, memory loss, fatigue, and scalp reactions.
Chemotherapy uses chemicals that intentionally damage the body’s cells with the expectation that healthy cells can more easily recover from the damage than tumor cells can. Treating an ependymoma or any brain tumor with chemotherapy is often ineffective because the chemicals can’t cross the blood-brain barrier, a border that protects the brain from potentially harmful foreign substances.
Chemotherapy is not often used to treat an ependymoma, but it may be recommended for tumors that can’t be treated effectively with surgery or radiation.
How Does Ependymoma Progress?
The prognosis for a person with an ependymoma depends on several factors. On average, the five-year survival rate for all types of ependymoma is about 84%. However, aggressive tumors or those that do not respond well to treatment can lower survival rates.
Some factors that impact the prognosis include:
- Age. People diagnosed at a younger age generally have better outcomes than those diagnosed later.
- Treatment effectiveness. Survival rates are better for those whose ependymomas can be removed entirely with surgery or respond well to treatment.
Ependymomas usually don’t spread to other parts of the body, but they can spread to other parts of the central nervous system via the cerebrospinal fluid (CSF).
How Is Ependymoma Prevented?
There is no clear way to prevent ependymoma from occurring. Even the lifestyle changes that can decrease the risk of many other types of cancer, such as quitting smoking or maintaining a healthy weight, may not reduce the chance of developing a brain tumor.
The only widely accepted preventative measure for brain tumors is the avoidance of high doses of radiation to the head.
Ependymoma Caregiver Tips
Caring for someone with a brain tumor can be even more challenging than the already high demands of caring for someone with any other type of severe and progressive illness. Along with the physical changes that make other cancers and serious illnesses so physically and emotionally exhausting to deal with, brain tumors also often produce psychological and cognitive changes in the patient that can also threaten the caregiver’s well-being.
As you care for your loved one through the progressive stages of their illness, keep these tips in mind:
- Learn as much as possible about the potential effects of your loved one’s specific type of brain tumor. This will allow you to understand how the illness affects the sufferer’s behavior.
- Get help from your friends and family. Caring for a brain tumor patient is a huge task, and you shouldn’t try to do it alone.
- Take time whenever possible to step away from the patient and the illness and find time for yourself. Acknowledge that it is normal and acceptable to need occasional relief from caregiving burdens.
- Find a support group. It can be beneficial to learn that you are not alone and that other people understand what you are going through.
Some people with ependymomas also suffer from other brain and mental health-related issues, a condition called co-morbidity. Here are a few of the disorders commonly associated with these tumors:
Ependymoma Brain Science
The ependymal cells from which ependymomas grow are a type of glial cell. Glial cells are part of the central nervous system’s support system. They are not nerve cells, but they help nerve cells live and work. Glial cells perform functions such as physically supporting nerve cells, bringing them oxygen and nutrients, and fighting off harmful foreign invaders.
Ependymal cells form a thin membrane that lines the spinal column and the ventricles, open spaces within the brain. These areas are where cerebrospinal fluid (CSF) is produced. CSF flows through the central nervous system, cushioning CNS tissues, providing them with nutrients, and removing waste. Ependymal cells are covered with tiny hair-like projections called cilia that wave in an organized pattern and help move the CSF from the ventricles and spinal column through the rest of the CNS.
Title: Study of Stored Tumor Samples in Young Patients With Brain Tumors
Principal investigator: Amar Gajjar, MD
St. Jude Children’s Research Hospital
This laboratory study looks at stored tumor samples in young patients with brain tumors. Studying tumor tissue samples from patients with cancer in the laboratory may help doctors learn more about changes that occur in DNA and identify biomarkers related to cancer.
The overall objective of this non-therapeutic protocol is to develop and molecularly characterize patient-derived orthotopic xenografts (PDOXs), organoids, and in vitro models derived from medulloblastomas, High-Grade Neuroepithelial Tumors (HGNET), CNS embryonal tumors, Atypical Teratoid Rhabdoid Tumors (ATRTs), Choroid Plexus Carcinomas (CPCs), ependymomas, and gliomas. The investigators will characterize the genome-wide mutation, expression, and epigenetic signatures of these models and compare them with the primary tumors from which they were derived, thus creating a well-characterized and invaluable resource for research on these rare and deadly pediatric brain tumors. This will also provide important insights into intratumoral heterogeneity and molecular abnormalities that may influence the selective pressures driving evolution and tumor growth, as in PDOXs, organoids, or in vitro cultures, and define the relationship between these abnormalities and tumor histologic and clinical characteristics. This objective will be achieved by applying state-of-the-art DNA, RNA, and epigenome analysis tools to the study of fresh-frozen and/or cryopreserved, fixed, and cultured tumor cells, PDOXs, and organoids. The establishment of patient-derived orthotopic xenografts, organoids, and cell cultures from each tumor sample will also allow in vitro and in vivo analysis of tumor cell growth, signaling, and therapeutic response.
Title: Memantine for Prevention of Cognitive Late Effects in Pediatric Patients Receiving Cranial Radiation Therapy for Localized Brain Tumors
Principal investigator: Heather M. Conklin, PhD
St. Jude Children’s Research Hospital
Children with brain tumors who have had radiation therapy are at risk for problems with attention, memory, and problem-solving. Such issues may cause difficulty in school and daily life. Memantine, the drug being used for this study, is not yet approved for use in children by the U.S. Food and Drug Administration. However, studies have shown some improvements in memory for patients with dementia, Attention Deficit Hyperactivity Disorder, and autism. Scientists have also used this medication for adult cancer patients receiving radiation therapy, with results showing less cognitive decline over time compared to patients taking a placebo (inactive pill). These studies have also shown few side effects.
This is a pilot/feasibility study and the first known study involving children with a cancer diagnosis or brain tumor.
- To estimate the participation rate in a study of memantine used as a neuroprotective agent in children undergoing radiotherapy for localized brain tumors (low-grade glioma, craniopharyngioma, ependymoma, or germ cell tumor)
- To estimate the rate of memantine medication adherence
- To estimate the rate of completion of cognitive assessments
- To estimate the effect size of change in neurobehavioral outcomes (cognitive, social, quality of life, neurologic) associated with memantine
- To evaluate the frequency and nature of memantine side effects as measured by the Systematic Assessment for Treatment Emergent Events (SAFTEE)
Title: Chemotherapy and Donor Stem Transplant for the Treatment of Patients With High-Grade Brain Cancer
Principal investigator: Kris M. Mahadeo
M.D. Anderson Cancer Center
This phase I trial investigates the side effects and effectiveness of chemotherapy followed by a donor (allogeneic) stem cell transplant when given to patients with high-grade brain cancer. Chemotherapy drugs, such as fludarabine, thiotepa, etoposide, melphalan, and rabbit anti-thymocyte globulin, work in different ways to stop the growth of tumor cells, either by killing the cells, preventing them from dividing, or by stopping them from spreading. Giving chemotherapy before a donor stem cell transplant helps kill cancer cells in the body and helps make room in the patient’s bone marrow for new blood-forming cells (stem cells) to grow. When the healthy stem cells from a donor are infused into a patient, they may help the patient’s bone marrow make more healthy cells and platelets and may help destroy any remaining cancer cells.