What Is an Ependymoma and How Is It Treated?

An ependymoma is a tumor of the central nervous system that affects more children than adults. We'll explain everything you need to know here.

Reviewed and approved by the doctor Diego Pereira.

Written by Carmen Martín

Last update: 05 June, 2023

Ependymoma is a type of tumor that forms in the central nervous system. It can develop in both the brain and the spinal cord. It’s more common in young children than in adults; however, it can arise at any age. Its presence tends to cause a blockage of cerebrospinal fluid circulation.

The problem is that many people may be asymptomatic for a long time or express very nonspecific symptoms. Hence, diagnosis is often delayed. Fortunately, there are now several treatments available to address the problem. In this article, we’ll tell you all the details.

What is an ependymoma?

Ependymoma is a primary tumor that can begin in the brain or spinal cord (central nervous system). It doesn’t come from any other part of the body. An article shared by National Center for Biotechnology Information explains that it develops from glial cells.

To be more precise, it comes from ependymal cells, those that line the ventricles of the brain and the ependymal duct of the spinal cord. The disease constitutes about 5% of intracranial gliomas in adults and up to 10% of childhood central nervous system tumors.

Ependymal cells are responsible for producing cerebrospinal fluid at the level of the cerebral ventricles. In the rest of the central nervous system, they facilitate the movement of this fluid. In itself, it plays an important role in protecting this system.

Ependymoma occurs when these cells proliferate uncontrollably, resulting in a tumor. It’s usually soft, gray, or reddish in color. And while it’s rare, it can affect people of any age. The main difference between children and adults is the location.

• In children, this types of cancer usually affects the lower half of the brain.
• Meanwhile, in adults, it’s more common for the spinal cord to be affected.

Types and grades of ependymoma

Ependymoma can be classified into different types and grades. The types are distinguished from each other according to their location in the brain or within the spine. On the other hand, the grades correspond to the atypia of the cells.

That is, cancer cells that differ slightly from normal cells constitute a low-grade tumor. However, those that are formed by cells that have very little resemblance to healthy cells will be high-grade.

Grade I ependymoma

Grade I ependymomas are those formed by cells that grow slowly. Within this group, we find the subependymoma and myxopapillary ependymoma types. Both types are more frequent in adults than in children.

• Subependymomas are those that develop near the cerebral ventricles.
• Myxopapillary ependymomas occur in the spinal cord. In fact, it usually affects the lower part of the spinal cord.

Grade II tumors

Grade II tumors are more frequent than the previous ones. They’re faster growing and include a multitude of subtypes. For example, they include papillary and cellular ependymomas.

Grade III ependymomas

Grade III ependymomas are the most aggressive. The cells are very atypical and have very rapid growth. Also called an ‘anaplastic ependymoma‘. It’s more common for them to develop inside the skull, either in the brain or in the brainstem.

They have a greater capacity for dissemination, as they can spread through the cerebrospinal fluid. It’s therefore possible that other tumor foci may appear in the brain or spinal cord. However, they don’t usually metastasize to other organs.

What are the symptoms?

Ependymoma occurs in different parts of the central nervous system. In addition, it can affect people of any age. All these factors make the symptomatology very variable. In fact, as we pointed out in the introduction, some people have hardly any symptoms at all.

One of the most frequent signs is headaches. The tumor can cause intracranial pressure to increase, so nausea, vomiting, and malaise are also common. Some patients experience blurred vision or loss of vision.

Loss of balance, difficulty walking, and seizures may also occur. When ependymoma affects the medulla, weakness, and tingling in the extremities are common. There may also be neck pain.

Causes and risk factors

As with any other tumor, ependymoma appears because the cells begin to proliferate in an uncontrolled manner. The cause is a mutation in the DNA of these cells. However, it isn’t known why this occurs.

However, it has been shown that people with neurofibromatosis type 2 are more at risk for this disease. This is a genetic disorder that favors the formation of tumors in the nervous system.

How is an ependymoma diagnosed?

The diagnosis of ependymoma is complex and requires certain complementary tests. Often, the neurologist performs a complete neurological examination to evaluate the patient’s senses, coordination, reflexes, and cranial nerves.

Through this examination and knowledge of the symptoms, the presence or absence of the tumor and its location can be determined. Imaging tests, such as magnetic resonance imaging or computed tomography, are usually performed at that time.

As the Mayo Clinic specialists explain, these tests are necessary to observe the tumor. In some cases, a lumbar puncture is also performed. This is a test that consists of extracting cerebrospinal fluid by puncturing a space between the vertebrae.

The fluid is analyzed in the laboratory to determine if there are tumor cells or alterations that indicate their presence. The tumor itself is also usually analyzed to see what the cells that make it up are like.

Treatments available

Fortunately, there are different ways to treat this disease. In fact, as explained in a publication by St. Jude Children’s Research Hospital the survival rate is quite high. The five-year survival rate is considered to be between 70 and 85%.

The first therapeutic option is surgery. The aim is to remove the entire tumor or to reduce its size as much as possible. The problem is that it’s usually located in delicate areas that are difficult to operate on. It’s therefore not always possible to remove the entire tumor.

Many patients, therefore, require other treatments to eliminate residual cancer cells. One of these is radiotherapy. There are different modalities, such as intensity-modulated or proton therapy.

These are techniques that aim to focus radiation on the tumor area and reduce damage to healthy tissues. Radiation therapy may be useful to prevent the recurrence of ependymoma. Radiosurgery, which consists of concentrating the radiation on specific points of the tumor, is also used.

Chemotherapy is another link in the treatment. However, it’s not very effective. This option is usually reserved for cases in which both surgery and radiation therapy have failed.

What to keep in mind

Ependymoma is a tumor that develops from certain glial cells. These cells are found in both the brain and the spinal cord. Therefore, it’s possible for this tumor to appear anywhere in both areas.

Although it’s considered a rare type of cancer, it’s important to be aware of its possible symptoms in order to identify it. The earlier a diagnosis is made, the more likely it is that treatment will be effective.