Ependymoma

Medically reviewed by Karen Gill, MD on October 9, 2017Written by James Roland on October 9, 2017

Overview

Your brain and spinal cord have spaces that contain cerebrospinal fluid. It’s a fluid that helps protect the brain and spinal cord from injury and from infection from toxins. These spaces are called ventricles when they are located in the brain and spinal cord. An ependymoma is a rare tumor that forms from cells in the lining of these spaces.

Depending on its location and aggressiveness, an ependymoma will be classified as one of three main types:

  • Classic. These are fairly well-defined tumors that usually appear in the lower part of the brain containing the cerebellum and the brain stem. They can also appear in the spinal cord or within the cerebral cortex, the upper part of the brain.
  • Anaplastic. These tend to be fast-growing tumors and often indicate a poor outcome. They also tend to develop in the area of the brain that contains the cerebellum and brain stem. This part of the brain is called the posterior fossa.
  • Myxopapillary ependymoma. These tumors are more common in adults than in children. They are usually limited to the base of the spine.

Causes and risk factors

Ependymomas are rare, with only about 200 new cases reported in adults and children in the United States each year. The tumors are much more common in children than in adults, with most tumors appearing in infants and young children.

As with most brain tumors, there are no known causes for ependymomas. Researchers have noted that ependymomas do tend to cluster in families, so there may be an inherited risk. Also, spinal ependymomas are more common in people with neurofibromatosis type 2 (NF2), a condition in which noncancerous tumors grow in the nervous system.

Symptoms

One of the main symptoms of ependymomas is pressure in the brain, which causes headaches. Often your feel these headaches when you first wake up in the morning. Children with ependymomas may also experience hydrocephalus, a buildup of fluid in the brain. This can cause increased head size in infants.

Other symptoms of ependymomas are:

  • nausea and vomiting (more frequent in the morning)
  • low energy
  • irritability
  • difficulty walking (an ependymoma can cause the eyes to cross, affecting balance)

About 90 percent of ependymomas in children are located in the brain. The majority of ependymomas in adults are in the spine. Spinal tumors can cause backaches and soreness and tingling in the legs.

Diagnosis

Diagnosing an ependymoma can require several tests. Your doctor will begin by reviewing your symptoms and medical history. You’ll have a physical examination that should include a test of your reflexes, coordination, eye and facial movement, and muscle strength.

Other tests include:

  • Magnetic resonance imaging (MRI). This is a noninvasive screening that uses a magnetic field and radiofrequencies to create images of the brain or any organ being screened. No radiation is involved.
  • Computed tomography (CT) scan. This is a special type of X-ray that produces cross-section images of the brain. These “slices” are more detailed than standard X-rays.
  • Biopsy. This is an invasive test. Once the doctor locates the tumor, they’ll remove a small portion of tissue surgically or with a needle, depending on the tumor’s location. The tissue is studied in a lab to determine whether it’s cancerous.
  • Lumbar puncture. Also known as a spinal tap, this procedure is also an invasive one. Your doctor will use a needle to withdraw a sample of spinal fluid to see whether cancer cells are moving through the nervous system.

Treatment

The first option for treating an ependymoma is neurosurgery. The goal is to remove as much of the tumor as possible. Sometimes the location of the tumor can make that impossible.

If hydrocephalus is present, the surgeon may implant a shunt in the brain to help drain excess cerebrospinal fluid. A shunt is a tube that carries the fluid from the brain. In most cases, the fluid is routed to the abdomen, where it’s absorbed harmlessly into the body.

The doctor may use radiation therapy to help shrink the tumor if surgery couldn’t remove it in its entirety. Highly targeted radiation beams can reach the tumor without harming tissue around it. The person may take chemotherapy medications prior to surgery to help shrink the tumor.

If the cancer has spread, radiation or chemotherapy may be the best option. The treatment will depend partly on the age of the person with the ependymoma. Other factors include the location of the cancer and the overall health of the person.

Recovery

Recovering from cancer treatment can be a long and challenging experience.

Chemotherapy can leave a person weak and sick to the stomach. Neurosurgery sometimes leaves people feeling worse for a while than they did prior to the operation. It’s not uncommon to feel tired, confused, weak, and dizzy.

If the surgery was a success, however, these feelings should subside over time.

Outlook

If the tumor can be removed completely, there is about a 65 percent cure rate. However, new ependymomas can develop later on. Recurrent ependymomas can be difficult to treat. Someone with a myxopapillary ependymoma tends to have a better prognosis than someone with a classic or anaplastic type of ependymoma. Adults tend to have a better prognosis than children.

However, with good treatment, about 82 percent of people who have an ependymoma survive at least five years. There are also ongoing clinical trials testing new treatments and looking at how long-term effects of treatments can be minimized.

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