Ependymoma tumors

Learning you have a rare brain tumor can raise lots of questions in your mind. You wonder what caused it and what to do next as you start to look for more information. Forming a partnership with your health care providers will support you on the journey through diagnosis and treatment.

What is an ependymoma?

An ependymoma is one of three main types of gliomas – primary tumors of the brain or spinal cord that can form at any age. Ependymomas are rare tumors that may be benign (noncancerous) or malignant (cancerous). They’re more common in children than adults. Children are more likely to have brain tumors while adults are more likely to have ependymoma tumors of the spine. These tumors occur more often in White people and in males.

Ependymoma tumors usually form in the ventricles of the brain – areas of the brain that contain cerebrospinal fluid (CSF). This fluid surrounds and cushions the brain and spinal cord. They grow in ependymal cells, which line the ventricles in the brain and the center of the spinal cord. Ependymoma tumors may spread through cerebrospinal fluid, but rarely spread beyond the brain or spine.

Ependymoma risk factors and causes

One identified risk factor for ependymoma tumors is an inherited cancer syndrome called neurofibromatosis type 2. However, not everyone who has the syndrome will develop an ependymoma.

The causes of ependymoma are unknown. There’s no known way to prevent them. Researchers continue to search for answers about ependymomas and other brain and spinal cord tumors.

Types of ependymoma tumors

Ependymoma tumors are categorized in different ways. Your doctor may describe your tumor based on the grading system below.

• Grade 1 tumors: Grow slowly and have two subtypes.
  • Subependymoma: Benign brain tumor in the ventricles.
  • Myxopapillary ependymoma: Grows slowly and tends to start at the bottom of the spinal cord.
• Grade 2 tumors: Grow a little faster and may be in either the brain or the spine.
• Grade 3 tumors: Cancerous, fast-growing and most often located in the brain. They can be in the spine, too.
  • Anaplastic ependymoma: Often grows near the base of the brain and it grows faster than other types.

Ependymoma symptoms

Symptoms may start suddenly, or they may start slowly and get worse over time. Symptoms depend on the location of your tumor. For example, tumors in the spine are more likely to cause back pain, while tumors in the brain are more likely to cause headaches.

Ependymoma symptoms are different for everyone and may include:

• Headache
• Nausea and vomiting
• Dizziness and balance problems
• Eye problems, such as double or blurry vision
• Weakness or numbness in an arm or leg
• Trouble walking
• Bowel or bladder problems
• Back pain that spreads to the arms or legs

Both cancerous and benign tumors can cause ependymoma symptoms when they grow and put pressure on nearby nerves or other tissues.

Ependymoma diagnosis

Ependymoma diagnosis can be difficult because it’s hard to tell the difference from other tumors. You’ll probably be referred to a neurologist since they specialize in diagnosing conditions that affect the brain and spine. Your care may also involve a neurosurgeon or a neuro-oncologist, who work with cancers that affect the nervous system.

Your first visit to a specialist will include a regular physical exam along with a neurological exam. They’ll gather information by testing things like your balance, coordination, range of vision, reflexes, and the strength of your arms or legs. Your doctor will ask about your symptoms and how they started.

Testing for ependymoma

Magnetic resonance imaging (MRI) testing will be ordered. It’ll probably include the use of a contrast dye to help find any cancerous cells because cancer cells absorb more dye than healthy cells.

Functional MRI (fMRI) may be used to see whether your tumor is near parts of the brain that affect important functions like speech and movement. A lumbar puncture may be performed to gather cerebrospinal fluid to see if it contains cancer cells.

Other testing may be ordered to help your Advocate Health Care team make an accurate diagnosis of your condition.

Ependymoma treatment

Surgery is often the first treatment for ependymoma tumors whether they’re benign or malignant. Your surgeon will remove as much of the tumor as possible to relieve symptoms. If the tumor isn’t completely removed, having removed most of it will make further treatments work better.

Your surgeon will also gather tissue for a biopsy to identify what kind of tumor you have and whether it’s cancerous. In addition to visual evaluation of the tissue sample, genetic testing can offer more insights about what kind of tumor you have and guide treatments.

Depending on the tumor and your health, radiation therapy may be recommended to destroy anything that’s left of the tumor. In some cases, chemotherapy is recommended. Cancer clinical trials may offer access to new medications used for chemo, targeted therapy or immunotherapy.

Beyond ependymoma treatment

After you’ve completed treatment, you’ll be monitored regularly to see if any tumors come back. We also provide continuing support through rehabilitation therapy, support groups and other means.