What Is Ependymoma? A Spinal Cord Tumor Explained: Grades, Symptoms and Treatment

Ependymoma is a tumor that arises from ependymal cells, which line the fluid-filled spaces of the brain (ventricles) and the central canal of the spinal cord. In children it is found more often in the brain; in adults it is most often in the spinal cord, where it is the most common tumor growing from within the cord itself. Many spinal ependymomas are well defined, and when they can be removed completely, long-term control is often achievable.

Where do ependymomas occur?

• Spinal ependymoma: inside the spinal cord, most often in the neck (cervical) or upper back (thoracic) region. Radiology reports describe it as an "intramedullary" tumor.
• Myxopapillary ependymoma: at the lower end of the cord, in the conus and the filum terminale, among the nerve roots of the lower back.
• Intracranial ependymoma: around the brain ventricles. In children it is most commonly in the posterior fossa near the fourth ventricle; it can also occur in the cerebral hemispheres (supratentorial).

This article focuses mainly on spinal ependymomas. For a broader overview see our article on spinal cord tumors.

Is ependymoma benign or malignant?

Ependymomas are graded under the World Health Organization classification of central nervous system tumors. The grade in your pathology report describes how the tumor behaves under the microscope:

• CNS WHO grade 2: most spinal ependymomas belong here. They grow slowly and usually have a border that can be separated from the surrounding tissue. In the current classification, myxopapillary ependymoma is also grade 2.
• CNS WHO grade 3: previously called "anaplastic ependymoma", this form grows faster and is more likely to recur. It is more common inside the skull.

The current classification also takes location and molecular features into account. For example, spinal ependymoma with MYCN amplification is recognised as a separate, more aggressive type. So the honest answer to "benign or malignant?" depends on grade, location and molecular type. Because the spinal canal is narrow, even a low-grade ependymoma can damage the cord as it grows, which is why tumors that cause symptoms are treated.

What causes ependymoma?

In most people no cause is identified. Spinal ependymomas are more common in people with neurofibromatosis type 2 (NF2), an inherited condition. There is no proven link with diet, stress or lifestyle.

Symptoms of spinal ependymoma

Because these tumors usually grow slowly, symptoms tend to build up gradually:

• neck or back pain, often worse at night,
• numbness, tingling or burning in the arms or legs,
• clumsy hands and difficulty with fine tasks when the tumor is in the neck,
• leg weakness and unsteady walking,
• changes in bladder or bowel control.

Myxopapillary ependymomas typically cause lower back pain and sciatica-like leg pain, so they are sometimes first treated as a slipped disc.

Symptoms of ependymoma in the brain

When a brain ependymoma blocks the flow of cerebrospinal fluid (hydrocephalus), it can cause morning headaches, nausea and vomiting, balance problems and visual disturbance. In young children irritability and loss of developmental milestones may be seen.

Diagnosis

Contrast-enhanced MRI is the key test. A spinal ependymoma typically appears as a well-defined, enhancing mass in the centre of the cord, often with cysts or signs of old bleeding at its upper and lower ends. Once an ependymoma is suspected, the brain and the whole spine are usually imaged to check for spread through the spinal fluid. The final diagnosis comes from pathological and, where relevant, molecular examination of the tissue removed at surgery.

Treatment

Surgery

For spinal ependymoma the cornerstone of treatment is removal of the tumor as completely as can be done safely, using microsurgery. Complete removal is the single factor that most reduces the risk of recurrence. During the operation the spinal cord's motor and sensory pathways are continuously monitored. Because many spinal ependymomas have a clear plane between tumor and cord, complete removal is often possible in experienced hands.

Radiotherapy

Radiotherapy may be recommended if tumor remains after surgery, if the tumor is grade 3, or if it recurs. The decision is made jointly by the neurosurgeon and radiation oncologist.

Chemotherapy

Chemotherapy has a limited role in ependymoma and is generally considered for selected patients with recurrent disease or when surgery and radiotherapy are not sufficient.

More about the surgical pathway is available on our spinal cord tumor treatment page.

Recovery and follow-up

After surgery inside the spinal cord, temporary numbness, altered sensation or weakness can occur; in most patients these improve over weeks to months, helped by physiotherapy. The best predictor of function after surgery is function before surgery, which is why treatment is ideally planned while symptoms are still mild. Ependymomas can recur years later, so long-term MRI follow-up is needed; the interval depends on grade and the extent of removal.

Prognosis

Outcome depends mainly on location (spine or brain), grade, molecular type and how completely the tumor is removed. Long-term tumor control is generally good for completely removed grade 2 spinal ependymomas. Grade 3 tumors and some intracranial subtypes need closer follow-up and additional treatment. A personal outlook can only be given once the pathology result and the post-operative MRI are reviewed together.

Conclusion

Ependymoma is the most common tumor arising within the spinal cord in adults, and because its symptoms build slowly it can be mistaken for a disc problem. Night pain, spreading numbness, clumsy hands and unsteady walking should be assessed. Prof. Dr. Ömür Günaldı is a neurosurgeon in Ataköy, Istanbul, who plans the diagnosis and microsurgical treatment of spinal cord tumors. This article is for general information only; individual diagnosis and treatment require examination, imaging and pathology.