Where do ependymomas form?

Ependymomas form in or near the ependymal cells that line the fluid-filled spaces of the central nervous system. In adults, roughly 75 percent occur in the spinal cord, most commonly in the cervical or thoracic spine. A smaller number occur in the brain, typically near the ventricles. In children, the pattern is reversed, with most ependymomas occurring in the brain.

Should I get a second opinion?

Yes, particularly given how rare adult ependymoma is. A specialist evaluation can confirm the diagnosis, review the molecular features, and clarify the full range of treatment options. Because the stakes of the initial surgery are high for long-term outcomes, getting the plan right from the start matters.

Ependymoma

Q: How is ependymoma diagnosed?

Diagnosis begins with MRI of the brain and spine with contrast. Because ependymomas can occasionally spread through the cerebrospinal fluid, a complete workup typically includes imaging of both the brain and the entire spine. Definitive diagnosis requires tissue obtained during surgery, which is then analyzed to determine the grade, specific type, and molecular features of the tumor.

Specialized care for rare brain and spinal tumors where the depth of surgical experience matters most.

Overview

What Is an Ependymoma?

Ependymoma is a rare type of tumor that begins in the cells lining the fluid-filled spaces of the brain and spinal cord. In adults, about 75 percent of ependymomas occur in the spinal cord, and the rest occur in the brain. Most are slow-growing, and outcomes are generally favorable when the tumor can be completely removed.

Common symptoms depend on where the tumor is located. Spinal ependymomas often cause new or worsening back or neck pain, weakness or numbness in the arms or legs, trouble walking, or changes in bladder or bowel function. Brain ependymomas can cause headaches, nausea, vision changes, balance problems, or seizures.

Treatment usually starts with surgery to remove as much of the tumor as possible, sometimes followed by radiation depending on the tumor’s grade and how completely it could be removed. Dr. Sherman has experience treating ependymomas at Rutgers Cancer Institute in New Brunswick, NJ.

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The information on this page is general educational information and is not medical advice. It does not create a doctor-patient relationship or reflect a treatment recommendation for any individual. Treatment decisions require an individual evaluation by a qualified physician.

Ependymoma symptoms depend almost entirely on where the tumor is located. Spinal tumors often cause symptoms that can be mistaken for more common problems like disc disease, and patients are sometimes treated for months or even years for back pain before the ependymoma is found. Brain ependymomas cause symptoms related to the specific area affected or to increased pressure in the skull.

Here’s what makes symptoms worth investigating.

New, persistent back or neck pain. Pain from a spinal ependymoma often starts gradually and may worsen over months. Pain that is worse at night or at rest, that doesn’t improve with the usual measures, or that is accompanied by neurologic symptoms warrants imaging.

Weakness, numbness, or tingling in the arms or legs. Spinal ependymomas can cause focal neurologic symptoms that progress over weeks to months. New or worsening weakness or sensory changes, especially on one side or in a specific pattern, should be evaluated.

Changes in bladder or bowel function. Spinal ependymomas in the lower spine, including myxopapillary ependymomas near the end of the spinal cord, can cause urinary urgency, frequency, retention, or incontinence, and changes in bowel function. These symptoms always warrant imaging.

Difficulty walking or loss of balance. New unsteadiness, stumbling, or trouble coordinating the legs can be caused by spinal ependymomas and warrant evaluation.

Persistent headaches. Brain ependymomas can cause headaches, particularly if they obstruct the normal flow of cerebrospinal fluid and cause pressure to build up inside the skull. Morning headaches that improve after getting up, or headaches with nausea or vomiting, should be evaluated.

Nausea and vomiting. Unexplained nausea and vomiting, particularly when associated with headaches, can indicate increased intracranial pressure from a brain ependymoma.

Vision or hearing changes. Brain ependymomas in specific locations can affect vision, hearing, or balance and warrant evaluation.

Seizures. New-onset seizures in an adult always warrant brain imaging.

When to treat it as an emergency. Rapidly progressive weakness or numbness, loss of bladder or bowel control, sudden severe headache, or loss of consciousness should be treated as an emergency. Go to the nearest emergency room rather than waiting for an appointment.

For most ependymoma symptoms, the pace of evaluation is measured in days to a couple of weeks. Ependymomas are generally slow-growing, but a new pattern of neurologic symptoms should be evaluated without extended delay.

Imaging

MRI is the standard imaging study for suspected ependymoma, and a complete workup typically includes imaging of both the brain and the entire spine. This is important because ependymomas can occasionally spread through the cerebrospinal fluid to other parts of the central nervous system, and identifying any additional sites of disease at the start changes treatment planning.

On MRI, ependymomas typically appear as well-defined masses that take up contrast, often located within or near the ventricles of the brain or the central canal of the spinal cord. Spinal ependymomas often show a characteristic pattern that an experienced neuroradiologist can recognize, though the imaging alone cannot confirm the diagnosis or grade.

Tissue Diagnosis and Molecular Profiling

Definitive diagnosis of ependymoma requires tissue, which is typically obtained during surgical removal of the tumor. A neuropathologist examines the tissue to confirm the diagnosis and determine the grade and specific type.

Under the 2021 WHO classification, ependymomas are categorized not only by their appearance under a microscope but also by their anatomic location and certain molecular features, including specific gene fusions and DNA methylation patterns. This molecular subclassification helps predict how the tumor is likely to behave and can influence treatment decisions. Not all centers perform the full molecular workup for ependymomas, which is one of the reasons evaluation at a comprehensive cancer center matters.

Cerebrospinal Fluid Evaluation

For patients with higher-grade ependymomas or brain ependymomas, a lumbar puncture may be performed after surgery to check for tumor cells in the cerebrospinal fluid. This helps determine whether the disease has spread beyond the main tumor site and affects decisions about whether radiation should be delivered to a larger area than just the original tumor location.

Ependymoma treatment is built around one core principle: complete surgical resection whenever it can be achieved safely. More than any other factor, how much tumor is removed at the first operation predicts long-term outcomes. Everything else in the treatment plan is shaped by whether complete resection was possible and by the specific type and grade of the tumor.

Surgery: The Foundation

Surgery is the primary treatment for ependymoma. The goal is gross total resection, meaning complete removal of all visible tumor, while preserving neurologic function. For spinal ependymomas, which make up the majority of adult cases, this often means a delicate operation in which the tumor is separated from the surrounding spinal cord tissue. Many spinal ependymomas have a relatively clear boundary with the surrounding spinal cord, which makes complete removal possible in experienced hands. For brain ependymomas, the feasibility of complete removal depends heavily on location.

For myxopapillary ependymomas in the lower spinal cord, the surgical approach aims for complete removal when possible, because this is associated with the best long-term outcomes. These tumors can be more challenging to remove completely than other spinal ependymomas because they often involve the nerve roots at the lower end of the spinal cord.

For supratentorial ependymomas in the brain, surgical approach depends on the tumor’s specific location and its relationship to critical structures.

In all cases, the experience of the surgical team matters significantly. Because adult ependymoma is rare, not every neurosurgeon has extensive experience with these specific tumors, and the learning curve is real.

Radiation Therapy

Whether radiation is recommended after surgery depends on the grade of the tumor and the completeness of the resection. For lower-grade spinal ependymomas that have been completely removed, radiation is often not needed, and surveillance alone is appropriate. For Grade 2 tumors that could only be partially removed, or for Grade 3 tumors regardless of the extent of removal, radiation is typically recommended. Radiation is usually delivered in small daily doses over several weeks, focused on the area of the original tumor, though larger areas may be treated if the disease has spread within the central nervous system.

Chemotherapy and Targeted Therapy

Chemotherapy has historically played a smaller role in ependymoma treatment than in other brain and spinal tumors. More recently, a clinical trial of dose-dense temozolomide combined with lapatinib, a targeted therapy, showed meaningful disease control in adults with recurrent ependymomas, and this combination is now considered an option for patients with recurrent disease. Research into additional targeted therapies is ongoing, particularly for ependymomas with specific molecular features.

Clinical Trials

Because adult ependymoma is rare, clinical trials are an important part of the treatment landscape and can offer access to newer approaches. Whether a trial is appropriate depends on the specific tumor type, the patient’s overall health, and what’s currently enrolling.

Surveillance

After treatment, long-term surveillance with periodic MRI is standard for all ependymomas. This is particularly important because ependymomas can recur years after the initial treatment, and early detection of recurrence allows for more options. The specific schedule of follow-up imaging depends on the tumor type, grade, and treatment received.

How the Plan Gets Made

Ependymoma treatment is a long-horizon undertaking, and the decisions made at the time of the initial surgery shape everything that follows. Complete surgical removal, when achievable safely, is the strongest predictor of long-term disease control. For patients where complete resection isn’t possible, or where the tumor is higher grade, the combination of radiation and ongoing monitoring becomes central to the plan. Rutgers Cancer Institute is the only NCI-designated comprehensive cancer center in New Jersey.

Treatments commonly used for this conditionBrain Tumor Surgery, Spine Tumor Surgery, Stereotactic Radiosurgery, and Clinical Trial Enrollment.

Technology

Using XR Technology To Advance Medical Care

Dr. Sherman integrates VR and AR visualization into surgical planning and patient education, giving patients a clearer picture of their diagnosis and the approach to treatment.

Common Questions About Ependymoma

For many patients with adult ependymoma, especially spinal tumors that can be completely removed at surgery, long-term disease control or cure is achievable. Extent of resection is the strongest predictor of long-term outcomes, and many patients with grade 2 spinal ependymomas who undergo gross total resection have excellent long-term survival. Higher-grade tumors and those that can only be partially removed have a higher chance of recurrence and typically require additional treatment.

Outcomes vary significantly by location, grade, and extent of surgical removal. Published outcomes for adult ependymoma are generally favorable in aggregate, particularly for spinal tumors that are completely resected. Outcomes for brain ependymomas tend to be somewhat less favorable than for spinal, and higher-grade tumors generally have less favorable outcomes than lower-grade tumors. Individual prognosis depends on factors that can only be assessed by a treating physician.

Adult ependymoma is rare, and extent of surgical resection is the strongest factor in long-term outcomes. A neurosurgeon who has experience with these specific tumors is more likely to achieve a complete resection safely, particularly for spinal ependymomas, which require careful separation from the surrounding spinal cord tissue. The rarity of the disease means that specialist experience is harder to come by and more valuable when found.

Yes, and for that reason long-term surveillance is standard for all ependymomas, regardless of grade. Recurrence can happen years after initial treatment, which is why regular follow-up imaging continues long after the original surgery. Lower-grade tumors that are completely removed have low recurrence rates. Higher-grade tumors and those that could only be partially removed have a higher risk of recurrence.

Next Step

Newly Diagnosed or Seeking a Second Opinion?

Dr. Sherman is accepting new patients at Rutgers Cancer Institute in New Brunswick, New Jersey. Whether you’ve just been diagnosed or you’re looking for another perspective on a treatment plan, a consultation is the first step toward understanding your options.

Schedule a Consultation 732-235-6333

Ependymoma

What Is an Ependymoma?

Symptoms

Diagnosis

Treatment

Using XR Technology To Advance Medical Care

Common Questions About Ependymoma

Newly Diagnosed or Seeking a Second Opinion?