Treatment of Ependymoma

An ependymoma is a rare tumor that occurs in the brain or spinal cord in approximately 4 out of 100,000 people. Brain ependymomas are more common among children, whereas spinal cord ependymomas occur more often in adults. Although the treatment of an ependymoma can be challenging, choosing an experienced neurosurgeon can greatly ease the journey.

Surgery is the primary method used to treat ependymomas, but radiotherapy and chemotherapy also have a role to play. Finding the right treatment strategy for your particular case is an important discussion to be had between you, your family, and your team of doctors.

The goal of surgery is to remove as much of the tumor as possible. This usually improves prognosis and future outlook of recovery. Success of the surgery depends on the tumor size and location, as well as the experience and technical skill of your neurosurgeon. For example, tumors located in deeper areas or neighboring critical nerves and blood vessels may be more difficult to completely remove. In such cases, you and the surgical team may decide to remove only part of the tumor to preserve the surrounding structures as much as possible.

Surgery for a brain ependymoma involves first receiving general anesthesia to ensure that you are unconscious and pain-free throughout the procedure. The duration of surgery is usually 4 to 6 hours, but the exact time depends on the complexity of the case. An incision will be made over the skin of the scalp before a part of the skull bone is removed. The dura mater – the tough outer covering of the brain – is cut to expose the tumor. The tumor is then carefully removed with specialized instruments while preserving as much of the surrounding healthy brain tissue, blood vessels, and nerves as possible. After the tumor is removed, the dura mater is stitched, the bone is put back in place, and the skin is closed with stitches. You will then be awakened from general anesthesia and transferred to the recovery room, where you will be closely monitored for a couple of days.

Surgery for spinal ependymomas follows the same overall procedure. After general anesthesia, you will be placed face down, and an incision will be made on your back. A few bony structures along the spine, called laminae, will be removed to gain access to the tumor. Once the dura mater surrounding the spinal cord is opened, the tumor can be accessed and as much as safely possible will be removed. The dura and skin will be closed with stitches.

After surgery, you will remain in the hospital for a few days so doctors can monitor your recovery. As with any surgery, there is risk of complications, including:

• Bleeding
• Infection
• Cerebrospinal fluid leak
• Difficulty speaking
• Difficulty with balance
• Weakness of the limbs
• Bowel or bladder disturbances

Choosing a neurosurgeon with extensive experience treating ependymomas will minimize the risk of complications. However, should complications occur, your team of doctors will help you manage them. The goal of surgery is usually complete tumor removal, but if there are tumor remnants, your doctor may recommend radiotherapy and sometimes chemotherapy as the next course of action.

Radiotherapy is typically used after surgery to prevent growth of remaining tumor cells. Radiotherapy uses high-energy radiation to damage the genetic material of rapidly dividing cells. During radiotherapy, beams of radiation are directed at the tumor site or cavity. The radiation damages the DNA inside the tumor cells, which stops them from dividing and growing. Over time, the damaged cells die and the tumor shrinks. Doctors use special machines to deliver the radiation to the right part of the body.

There are different types of radiotherapy methods to treat ependymoma. In general, radiotherapy can be targeted precisely to the boundaries of the tumor. In some cases, even the intensity of beams can be fine-tuned to deliver the highest doses to the densest parts of the tumor.

Radiation therapy can also be administered in different ways. Fractionated radiotherapy involves multiple treatment sessions, typically lasting 20 to 30 minutes each. Conversely, stereotactic radiosurgery delivers a high dose of radiation in a single session or several sessions, often taking about an hour. 

Sometimes the ependymoma may have spread to other parts of the brain or spine after surgery. When this occurs, patients undergo craniospinal irradiation. Craniospinal irradiation is a treatment that delivers high-energy radiation beams to the entire brain and spinal cord. During the treatment, the patient lies on a table and a machine called a linear accelerator is used to direct radiation beams to the head and spine. The treatment is typically given in small doses over the course of several weeks.

Radiotherapy can cause inflammation, which may make symptoms worse. Doctors prescribe medicine called steroids to control this inflammation. Other side effects of radiotherapy treatment include changes to the skin (swelling, flaking, or redness), feeling sick (nausea), dry mouth, mouth irritation, and hair loss. Patients might feel tired, have trouble swallowing, or have changes in their sense of taste. There can also be short- and long-term problems with cognition and memory. Most of these side effects can be managed with help from your team of doctors.

Chemotherapy is a cancer treatment that uses drugs to kill cancer cells. Chemotherapy drugs work by stopping the cancer cells from growing and dividing, which can help shrink the tumor or stop it from spreading. Chemotherapy can be given in different ways – through a vein (intravenously), by mouth (orally), or even as an injection – depending on the type of cancer and your health. Certain chemotherapeutic drugs such as temozolomide, procarbazine, lomustine, and vincristine have been used to treat ependymomas, although their success has been limited. There are currently clinical trials ongoing to look for more effective chemotherapeutic drugs.

The wait-and-see approach is an alternative to active treatment for ependymoma. This involves regular monitoring through imaging tests like an MRI to detect any tumor growth. This approach may be favorable for older patients with low-grade (e.g., Grade 1) ependymomas who are not experiencing symptoms. Although observation without active treatment can be a viable option, it does come with some risks, such as the potential for the tumor to grow between scheduled imaging tests and invade nearby structures. However, this risk is low for low-grade tumors, as they tend to grow slowly.

If you or someone you know has been diagnosed with ependymoma, the treatment can be a challenging experience. It's normal to feel scared, overwhelmed, and uncertain about what to expect. Although the steps may be difficult, remember the treatment is intended to help you fight the cancer and regain your health. Your medical team will work with you to ensure you are as comfortable as possible during treatment, and they will provide you with support and guidance throughout the process. With time and patience, you can overcome this obstacle and move forward toward a brighter future.

• Surgery provides the best treatment option for ependymomas with the aim to remove as much of the tumor as possible.
• Radiotherapy is usually given after surgery to kill any remaining tumor cells.
• Currently, chemotherapy has been used with limited success in treating ependymomas.

• Collaborative Ependymoma Research Network (CERN)

• American Brain Tumor Assocation

• The Childhood Brain Tumor Foundation

• Pediatric Brain Tumor Foundation