An ependymoma is a type of cancerous tumor that forms either in the brain or the spinal cord. This type of tumor affects children more often than adults.

Ependymomas are the third most common type of brain tumor in children, affecting about 250 children in the United States each year. They are diagnosed most often in children ages 8 and younger. Adults with ependymomas are often diagnosed in their 30s or 40s. Ependymomas in children typically develop in the brain. By contrast, most adults diagnosed with the condition have tumors that develop in the spinal cord.

An ependymoma can be treated, although the treatment’s effectiveness depends on the tumor’s location within the brain or spinal cord. The best outcomes occur when all or most of the tumor is removed.

Ependymoma is a tumor that arises within the brain or spinal cord. It’s more likely to appear in children than adults.

Within the brain, an ependymoma typically forms near the ventricles, or open spaces, in the brain and around the spinal cord. Sometimes, the formation of an ependymoma blocks cerebrospinal fluid from flowing freely within the brain. This can lead to hydrocephalus, a condition in which cerebrospinal fluid builds up in the ventricles, putting pressure on the brain within the skull.

An ependymoma can develop within the central nervous system (the brain and spinal cord) when certain cells multiply uncontrollably. The cells are either ependymomal, which help create cerebrospinal fluid, or glial, which provide support to neurons and nerve cells.

Doctors don’t yet know what causes ependymoma. Most cases of ependymoma occur sporadically, with no confirmed cause.

However, certain inherited conditions have been linked to this form of cancer, most notably neurofibromatosis type 2.

People who have an ependymoma in the brain can experience:

• Headaches
• Nausea and vomiting
• Weakness
• Fatigue
• Irritability
• Loss of appetite
• Trouble concentrating
• Mood changes
• Personality changes
• Vertigo
• Blurry vision
• Cognitive impairment
• Falling behind on developmental milestones (for babies)
• Loss of coordination
• Balance problems
• Difficulty walking
• Seizures
• Hydrocephalus

If an ependymoma is located in the spinal cord, symptoms can include:

• Neck stiffness
• Neck weakness
• Neck or back pain
• Muscle weakness in one or both legs
• Bladder and bowel dysfunction or loss of control

Because doctors are unsure what causes ependymoma, they often do not know what circumstances increase the risk for this type of cancer.

However, some inherited conditions may increase the risk of ependymoma, including:

• Neurofibromatosis type 2
• Turcot syndrome B
• MEN1 syndrome

Doctors can diagnose ependymoma after learning about a patient’s medical history, performing a physical exam, and offering diagnostic tests that confirm the presence of a tumor. If an ependymoma is suspected based on imaging, a biopsy is required to confirm the diagnosis.

When patients are young children, doctors rely on parents to share information about their children’s symptoms and medical history. Doctors will ask how long their symptoms have occurred and whether there is a family history of cancer or brain tumors.

During a physical exam, neurological and/or cognitive tests to assess brain function will be performed. Doctors also look for signs of hydrocephalus.

The following tests may be used to diagnose an ependymoma:

• CT scan or MRI with or without contrast: An imaging exam that allows doctors to see tumors, growths, or abnormalities in the brain or spinal cord.
• Spinal tap (lumbar puncture), in which a needle is inserted into the spinal canal to remove cerebrospinal fluid for analysis. This can confirm the presence of an ependymoma.
• Biopsy, in which doctors remove a small amount of the tumor to determine if the cells are cancerous. If possible, doctors may opt to remove the entire tumor rather than a portion, and then biopsy it after surgery.

Surgery is typically recommended as the first-line treatment, except when the tumor is too large to be removed. In those cases, a patient may receive chemotherapy to shrink the tumor, followed by its surgical removal.

Radiation therapy may be used after surgery to destroy any remaining cancer cells. For children under 12 months old, however, radiation therapy is typically not offered.

Chemotherapy after surgery is generally not recommended—or helpful—for patients with an ependymoma. However, some doctors recommend chemotherapy for patients under 12 months old until they are old enough for radiation therapy.

About 79% of people diagnosed with ependymoma live for 5 years or longer. Patients tend to have better outcomes when doctors are able to remove all or most of the tumor surgically.

“Yale’s Pediatric Neuro-Oncology program is a multidisciplinary collaboration between pediatric neuro-oncologists, neurologists, neurosurgeons, neuro-radiologists, and neuro-pathologists specifically trained in the treatment of children with brain tumors,” says Smilow Cancer Hospital hematologist and oncologist Asher Marks, MD. “They have access to cutting-edge technologies, such as intra-op MRI and proton beam therapy, as well as the latest clinical trials to offer patients the best and most up-to-date treatments.”