Ependymoma

Ependymoma symptoms may be generalized or specific to the location and size of the tumor. They can be subtle. Some common symptoms include:

• Headaches (persistent, increasing in intensity over time, may wake a person up at night)
• Vomiting
• Seizures (new onset)
• Memory problems
• Blurred vision
• Mood disturbances
• Changes in personality
• Fatigue
• Balance problems
   

Symptoms of spinal ependymomas may include:

• Back or neck pain
• Weakness or numbness in the arms or legs
• Difficulty with coordination or balance
• Bladder or bowel dysfunction
• Changes in sensation

Most ependymomas are sporadic tumors that arise in people without known risk factors. 

• Exposure to Ionizing Radiation. People who have had radiation therapy (ionizing radiation) to treat cancer have an increased risk of developing ependymoma, especially those who received radiation treatment to their head and neck during childhood.
  
  Other forms of radiation, such as those from power lines, cellular phones, and microwave ovens, have not been shown to increase the risk of ependymoma.
  
  
• Family history. Ependymoma rarely runs in families, but some diagnosed cases may have a genetic component.
  
  Some rare hereditary cancer syndromes, like Li-Fraumeni syndrome and neurofibromatosis, are characterized by the early onset of multiple tumors, including ependymomas.
  
  Some inherited gene variants have been associated with a small increased risk of ependymomas. Inheriting one of these "risk variants" does not necessarily mean the person will develop ependymomas. Additional mutations are still required to lead to tumor formation.

• Neurological Examination. A thorough evaluation of the nervous system includes assessment of: mental status (awareness, memory), cranial nerves (nerves responsible for eye movement, taste, facial expressions), speech (speaking and understanding), muscle strength and grip, sensory function (ability to feel light touch, vibrations, temperature changes), balance and coordination, and reflexes
• Imaging.  Magnetic resonance imaging (MRI) is often taken before and after the administration of intravenous contrast to visualize the tumor.  In addition to imaging the brain, the spine is also checked  for ependymomas can migrate to sites throughout the spine.  Other imaging tests may be used to evaluate the tumor or help plan treatment, including computerized tomography (CT) scan, positron emission tomography (PET), functional MRI, perfusion MRI, and magnetic resonance spectroscopy.
• Lumbar puncture. Also known as a spinal tap, a lumbar puncture is a medical procedure in which a needle is inserted into the lower part of the spinal column to collect a sample of cerebrospinal fluid (CSF). A neuropathologist will test the CSF sample to look for tumor cells or other abnormalities.
• Surgery. When the history, neurological examination, and images are concerning for an ependymoma, the next step is to obtain diagnostic tissue. A tissue sample is required to establish the diagnosis and accurately classify the glioma. Tissue can be obtained during open surgery or by stereotactic biopsy.  Both procedures are performed in the operating room, usually under general anesthesia.
  
  

Histology

Pathologists use a microscope and special stains to identify the type of glial cell in the tumor sample.

Ependymal cells line the ventricles of the brain and the center of the spinal cord. These cells produce cerebrospinal fluid (CSF), which surrounds and protects the brain and spinal cord. Ependymal cells give rise to ependymomas.

Pathologists also look for signs of tumor malignancy (aggressiveness), such as:

• Irregular cellular appearance
• Cells actively multiplying
• Formation of new blood vessels
• Necrosis (dead tissue)
   

Molecular Analysis

The presence of specific genetic markers (mutations) has been shown to be a reliable predictor of tumor behavior and treatment response.

Examples of genetic markers relevant in categorizing ependymoma include:

• MYCN amplification of spinal cord ependymomas. Tumors with this biomarker tend to show rapid progression, early metastases, and resistance to treatment.
   

• Genetic markers provide clues about the tumor and allow physicians to individualize the treatment plan.
  

Grading

Pathologists also consider the cellular appearance and presence of genetic markers to assign a grade to the tumor. Grading is a prediction of how the cancer would behave without treatment. In ependymoma, a grade is assigned within a particular subtype and can range from 1 (less aggressive) to 3 (more aggressive).

A cure has not yet been identified for ependymoma. Surgical resection, radiation therapy, and chemotherapy are the primary treatment options. The location, size, and subtype of ependymomas determine which combination of treatments is best. 

If surgery is possible, aggressive resection can change the course of this disease for patients.  In general, a combination of surgery, chemotherapy, and radiation are often required.  Patients can do well from an oligodendrogliomas.  If the spine is involved, additional spinal radiation may be beneficial.

Learn more about treatment options >

Symptom Management

Treatment also includes managing symptoms caused by the ependymoma.

People who experience seizures are treated with an antiseizure drug, such as levetiracetam. Tumor-induced seizures can be challenging to treat, and surgical resection may be able to reduce seizure activity.

Glucocorticoids (steroids) can improve headaches and neurologic deficits caused by cerebral edema. Dexamethasone is frequently prescribed for this purpose but is associated with significant side effects and may shorten survival. Glucocorticoids are used at the lowest effective dose and, ideally, only until other treatments are planned.

Treatment Innovations

Treatment options for ependymoma have expanded in recent years. Some recent treatment advances include:

• Tumor treating electrical fields (Optune): A device worn on the scalp delivers alternating electrical fields to prevent the growth and division of cancer cells.
• Convention-enhanced delivery – Chemotherapy is slowly and continuously delivered to the tumor via a pump.
• Nanoparticle therapy – Special particles allow chemotherapy drugs to cross the blood-brain barrier and improve access to the tumor.
   

Learn more about treatment innovations at Stanford >

Clinical Trials

Ongoing research is needed to improve our understanding of brain tumors and develop more effective therapies. At Stanford, we are exploring new approaches to treating ependymoma, which may offer hope for patients in the future. Some treatments currently being studied are:

• Immunotherapies. Immunotherapy is a type of treatment that uses the patient's immune system to fight cancer. Some experimental immunotherapies for brain tumors include checkpoint inhibitors, CAR T-cell therapy, and vaccines.
• Molecular targeted therapies. Targeted therapy is a type of treatment that targets specific molecules or pathways involved in cancer growth and survival. Some experimental targeted therapies for brain tumors include drugs that target mutations in the IDH1 and IDH2 genes.
   

Patients may participate in clinical trials to access new treatments under investigation. Please check with your physician to see if you are eligible for a clinical trial.

 Learn more about our open clinical trials >