What Is an Ependymoma?

An ependymoma is a rare tumor of the brain or spinal cord. This type of tumor can spread throughout the central nervous system (CNS) and, in rare cases, to other parts of the body. Ependymomas can occur in children and adults, but they are more common in children.

This article will provide an overview of ependymomas, as well as their causes, diagnosis, treatment, and prognosis.

Types of Ependymoma

There are four grades, or types, of ependymomas, including:

1. Subependymomas (grade one): A slow-growing tumor near the ventricles in the brain. This type mostly affects adults.
2. Myxopapillary ependymomas (grade one): A slow-growing tumor in the lower spinal cord.
3. Ependymomas (grade two): The most common type of ependymoma. They grow faster than grade one tumors.
4. Anaplastic ependymomas (grade three): Fast-growing tumors that grow at the base of the brain and often reoccur after removal.

In most cases, grade one tumors grow more slowly and are less aggressive than grades two or three. A grade three ependymoma is the most aggressive and has a higher risk of recurrence.

Symptoms

Symptoms of an ependymal tumor depend on the type of tumor and its location in the body. A tumor pressing against the brain could affect physical movement in the area it controls, or cause weakness on one side of the body.

Symptoms may develop quickly or take years to evolve.

Some common symptoms of an ependymal tumor include:

• Headache
• Backpain
• Nausea
• Vomiting
• Dizziness
• Weakness or numbness in the limbs
• Bladder or bowel issues

If you are experiencing these symptoms, contact your physician for an examination or testing.

Causes

Ependymomas form from glial cells that grow abnormally. Glial cells support functions of the central nervous system. When these cells grow out of control, they can form ependymomas.

People with a genetic disease called neurofibromatosis (genetic disorders causing tumors to form on nerve tissue) have a significantly increased chance of developing an ependymoma.

There is currently no known way to prevent an ependymal tumor. There is a genetic component to this type of cancer that influences development and progression of cancer cells.

Diagnosis

Diagnosing ependymomas depends largely on the type of tumor.

Because this type of cancer starts in the brain or spinal cord, a physician will likely perform a neurological exam as the first step in a diagnosis. This involves assessing brain function by examining:

• Hearing
• Vision
• Balance
• Coordination
• Reflexes
• Muscle strength

If your physician suspects a tumor, the doctor will then order imaging or other tests to assist in a diagnosis of ependymoma, including:

• Magnetic resonance imaging (MRI): Noninvasive imaging test used to retrieve multidimensional images within the body.
• Computerized tomography (CT) scan: A series of noninvasive X-rays taken in slices to capture images within the body.
• Biopsy: Removing cells within the tumor to be sent to a lab for analysis.
• Lumbar puncture (LP): The collection of a small amount of cerebrospinal fluid to test for cancer cells.

Proper diagnosis is essential to see if a tumor has metastasized (spread) to other parts of the central nervous system or spread to other parts of the body.

Treatment

The most common treatments for ependymoma include:

• Surgical removal: Surgery is the primary treatment option for ependymomas. The goal is to remove the entire tumor or as much of it as possible.
• Chemotherapy: If a tumor is unable to be removed or is only partially removed, or if it has metastasized to other parts of the body, chemotherapy may be an option.
• Radiation: This treatment uses high-dose radiation to shrink tumors and destroy cancer cells. 

Discuss these options with your physician to understand the risks and benefits of your treatment plan.

Prognosis

The prognosis for patients diagnosed with ependymoma depends on several factors, mainly the type and location of the tumor, the patient's age and prior medical history, as well as the interventions used and how well they respond to treatment. Tumor mutations can also be a factor in prognosis.

The overall five-year survival rate for ependymoma is about 84%. The overall five-year survival rate for children is about 65%. But again, these rates can vary widely based on the previously mentioned factors.

Prognosis will be specific to the individual. If you are diagnosed with ependymoma, speak with your physician to get a clear understanding of your individual prognosis.

Summary

Ependymomas are a rare type of brain tumor occurring in the central nervous system. Early diagnosis is essential to detect a tumor and begin treatment. If you are experiencing unusual neurological symptoms, make an appointment with your physician.

A Word From Verywell

Being diagnosed with an ependymoma can be frightening. However, there are treatment options available, and in many cases, the survival rate is high. It's important to discuss your diagnosis thoroughly with your physician and develop an effective treatment plan.