Ependymoma is a rare type of brain or spinal cord tumor that starts in ependymal cells, which line the fluid-filled spaces of the brain and the central canal of the spinal cord. These tumors can occur in both children and adults, but the location, behavior, and outlook often differ by age. Treatment typically involves surgery to remove as much of the tumor as possible, often followed by radiation therapy, and the prognosis depends heavily on the tumor’s grade, location, and how completely it is removed.
What Is an Ependymoma Exactly?
Ependymomas arise from ependymal cells. These are the cells that line the ventricles of the brain and the central canal of the spinal cord. They help produce and circulate cerebrospinal fluid (CSF), the fluid that cushions the brain and spinal cord.
Most ependymomas are slow-growing. However, some are more aggressive and can spread through the CSF pathway to other parts of the brain or spine. The World Health Organization (WHO) classifies ependymomas into grades 1, 2, and 3 based on how abnormal the cells look under a microscope. Grade 1 tumors are the least aggressive, while grade 3 tumors are the most aggressive.
Ependymomas are not common. They make up a small percentage of all brain tumors. In children, they are more often found inside the brain, particularly in the back of the brain near the cerebellum. In adults, they are more frequently found in the spinal cord.
What Are the Symptoms of Ependymoma?
Symptoms depend on where the tumor is located. Because the tumor takes up space inside a fixed space (the skull or the spinal canal), symptoms often come from increased pressure or from the tumor pressing on nearby structures.
For tumors in the brain, common symptoms include:
- Headaches, especially in the morning
- Nausea and vomiting
- Blurred or double vision
- Problems with balance and coordination
- Dizziness
- Seizures
- Changes in behavior or personality
For tumors in the spinal cord, symptoms may include:
- Back pain
- Weakness or numbness in the arms or legs
- Trouble walking
- Loss of bladder or bowel control
These symptoms can develop slowly over months. In children, symptoms may be harder to notice because young children cannot always describe what they feel. Parents may notice a child becoming clumsy, having trouble in school, or complaining of headaches that get worse over time.
What Causes Ependymoma?
The exact cause is not known. Most ependymomas occur without any clear risk factor or family history. There is no proven link to lifestyle choices, diet, or environmental exposures that a person can control.
A small number of cases are linked to genetic conditions. For example, people with neurofibromatosis type 2 (NF2) have a higher risk of developing spinal ependymomas. But most people diagnosed with ependymoma do not have this or any other known genetic syndrome.
Research is ongoing to understand the genetic changes inside tumor cells that drive their growth. Scientists have identified certain molecular subtypes of ependymoma that behave differently and respond differently to treatment. This is an active area of study, and molecular testing is increasingly used to help guide treatment decisions.
How Is Ependymoma Diagnosed?
Diagnosis begins with a neurological exam. The doctor will check reflexes, balance, coordination, vision, and strength. If a brain or spinal tumor is suspected, imaging is the next step.
Magnetic resonance imaging (MRI) is the standard imaging test. It provides detailed pictures of the brain and spinal cord and can show the size and location of the tumor. A spinal MRI is often done as well to check whether the tumor has spread through the cerebrospinal fluid.
The only way to confirm an ependymoma diagnosis is with a biopsy. This means a surgeon removes a sample of the tumor tissue, and a pathologist examines it under a microscope. The pathologist determines the tumor grade and, increasingly, runs molecular tests that help predict behavior.
A lumbar puncture, also called a spinal tap, may be done to check for tumor cells in the cerebrospinal fluid. This helps determine whether the tumor has spread along the CSF pathway.
What Are the Treatment Options for Ependymoma?
Surgery is the first and most important treatment. The goal is to remove the entire tumor safely. Studies have consistently shown that the extent of surgical removal is the strongest predictor of outcome. A complete removal, called a gross total resection, is associated with better outcomes than a partial removal.
Whether the surgeon can remove the whole tumor depends on its location. Tumors in the spinal cord may be easier to remove completely than tumors in certain parts of the brain where nearby vital structures are at risk.
After surgery, treatment depends on the tumor grade, the extent of removal, the patient’s age, and whether the tumor has spread.
- Radiation therapy is often used after surgery, especially if the tumor was not completely removed or if it is a higher-grade tumor. It is also used for tumors that come back after initial treatment.
- Chemotherapy is used in certain situations. It is more commonly used in young children, because radiation can harm the developing brain. In adults, chemotherapy is generally used for recurrent tumors that do not respond to other treatments.
- Clinical trials may be an option. Researchers are studying newer drug therapies, including targeted therapies that attack specific genetic changes in tumor cells.
For adults with a completely removed, low-grade ependymoma, some doctors recommend careful monitoring without immediate radiation. This is a decision made on a case-by-case basis, and the evidence supporting observation versus immediate radiation is still evolving.
What Is the Prognosis for Ependymoma?
Prognosis is a medical estimate of how a disease is likely to progress. It is not a guarantee. For ependymoma, several factors shape the outlook.
The most important factor is whether the tumor can be completely removed with surgery. Patients who have a gross total resection have better outcomes than those who have residual tumor left behind.
Tumor grade matters. Grade 1 tumors, such as subependymomas and myxopapillary ependymomas, grow very slowly and have an excellent prognosis after complete surgical removal. Grade 2 tumors are intermediate. Grade 3 tumors, called anaplastic ependymomas, grow more quickly and have a higher chance of recurrence.
Location also matters. In adults, spinal ependymomas generally have a better prognosis than intracranial ones. In children, tumors in the back of the brain (posterior fossa) are the most common and are often challenging to treat because of their location near critical brain structures.
Age at diagnosis is relevant. Young children, especially infants, tend to have a worse prognosis than older children and adults. This is partly because treatment options are more limited for very young children due to the risks of radiation to the developing brain.
Overall, five-year survival rates for ependymoma vary widely depending on these factors. Some studies report five-year survival rates above 80 percent for patients with completely removed low-grade tumors. For higher-grade or incompletely removed tumors, the rates are lower. These are population-level statistics, not individual predictions.
Can Ependymoma Come Back After Treatment?
Yes. Ependymoma can recur, even after successful treatment. The risk of recurrence is highest in the first few years after treatment, but it can happen many years later. This is why long-term follow-up is essential.
Regular MRI scans are typically done every few months for the first few years, then less frequently over time. The exact schedule depends on the tumor’s grade, location, and treatment history.
When ependymoma recurs, treatment options depend on where it comes back, how much time has passed, and what treatments were used before. Surgery is often attempted again if possible. Radiation may be used if it was not given before, or if enough time has passed since the previous course. Chemotherapy is another option, but its effectiveness for recurrent ependymoma is limited.
What Is the Follow-Up Care After Ependymoma Treatment?
Follow-up care has two main goals: monitor for recurrence and manage the long-term effects of treatment.
Neurological exams and imaging are routine. The frequency of scans is individualized, but most patients have an MRI every three to six months for the first few years after treatment.
Long-term effects depend on the tumor location and the treatments used. Radiation to the brain can affect cognitive function, especially in children. It can also cause hormonal problems if the radiation field includes the pituitary gland. Surgery near the spinal cord can cause weakness, numbness, or bladder and bowel issues.
Many patients benefit from rehabilitation services, including physical therapy, occupational therapy, and speech therapy. Neuropsychological testing can help identify cognitive changes and guide support strategies.
Survivors of ependymoma need lifelong follow-up with a medical team that understands their specific treatment history. This is especially true for children treated at a young age, as new late effects can emerge years after treatment.
Frequently Asked Questions
Is ependymoma a cancer?
Yes, ependymoma is a type of cancer, though it is often slow-growing. Its behavior ranges from low-grade, slow-growing tumors to high-grade tumors that grow quickly and are more likely to recur.
Can ependymoma be cured?
Complete surgical removal can cure some ependymomas, especially low-grade tumors that are fully resected. Higher-grade tumors and those that cannot be fully removed are harder to cure and may require additional treatment.
How fast does an ependymoma grow?
Growth rate depends on the tumor grade. Low-grade ependymomas grow slowly over years, while high-grade anaplastic ependymomas grow more quickly, sometimes within months.
Is ependymoma hereditary?
Most ependymomas are not hereditary. A small minority of cases are linked to genetic conditions like neurofibromatosis type 2, but the vast majority occur without any family history.
