Fast Facts

The CERN Foundation is dedicated to helping you understand ependymoma. See below for a list of ependymoma facts about the disease.

Ependymoma tumor description

• An ependymoma is a rare type of primary brain or spinal cord tumor
• An ependymoma is a rare type of glial tumor; glial cells are the cells that support brain
• Primary brain and spinal cord tumors are those that start in the central nervous system (CNS)
• These ependymoma tumors are classified and graded according to their appearance when viewed through the microscope
• Ependymomas can occur in children and adults

Ependymoma tumor incidence

Ependymoma tumors account for two percent (in adults) and five percent (in children) of CNS tumors.

Incidence in children:

• The third most common pediatric brain tumor
• The most common type of primary spinal cord tumor
• Approximately 250 children are diagnosed per year
• Most commonly occur in the brain

Incidence in adults:

• Two percent of adult primary brain and spinal cord tumors
• Approximately 1,100 adults are diagnosed per year
• Most commonly occur in the spinal cord

Staging and grading

• Ependymoma may spread throughout the CNS
• Spread outside of the CNS is rare
• All can recur (regrow)
• In the World Health Organization (WHO) Classification of Central Nervous System Tumors, ependymal tumors are classified based on anatomic location (tumor location), histology (grading) , and molecular features (subgroups) . The 2021 revision of the WHO classification 5th edition, further refines several subgroups based on molecular genetic features. Read the CERN Foundation Supplement to NBTS Blog Post ‘What You Need to Know About the New Updates to Brain Tumor Classification and Grades’ for more information.
• Graded on a scale of I to III (World Health Organization [WHO] Grading system)
• Grade I
  • Myxopapillary ependymoma and subependymoma
  • Myxopapillary ependymomas generally occur in the spinal cord in adults
  • Both tumors tend to grow slowly, especially the subependymoma
• Grade II and III
  • Classic ependymomas and ependymoma subtypes or variants (Grade II) and anaplastic ependymoma (Grade III)
  • These occur mainly in the brain
  • These tumors grow more quickly than Grade I tumors
• Molecular (genetic) testing of ependymomas has determined that there are 10 distinct types of tumor

Spinal cord and brain tumor symptoms

Symptoms depend on the tumor location. Any symptoms you have should be discussed with your doctor

Common brain tumor symptoms include:

• Headache or pressure in the head
• Nausea or vomiting
• Blurred vision
• Weakness or numbness and tingling

Common spinal cord symptoms include:

• Back pain
• Weakness in the arms or legs
• Numbness or tingling in the arms, legs or trunk
• Problems going to the bathroom or controlling bowel or bladder function

Causes and treatment

• The cause is not known. It rarely occurs in multiple family members, although does occur in people with Neurofibromatosis Type 2 (NF2).
• Most people with ependymomas, of any grade, will undergo surgery to remove a tumor.
• Depending on the tumor grade, location and how much of the tumor has been removed, radiation therapy is often given.
• The role of chemotherapy is limited, although some new regimens are promising. Researchers are working to find new effective treatments.