Fast Facts
The CERN Foundation is dedicated to helping you understand information about ependymoma. See below for a list of ependymoma facts about the disease.
Ten Facts about Ependymoma
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Ependymoma is a primary central nervous system (CNS) tumor that starts in the brain or spine.
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Ependymomas develop from ependymal cells. Ependymal cells are glial cells that support the brain. Ependymoma is a type of primary glioma tumor.
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Ependymomas occur in both adults and children.
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Symptoms related to an ependymoma depend on the location of the tumor.
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Less than 2% of CNS tumors in adults are ependymoma, approximately 1,100 adults are diagnosed with ependymoma per year in the US.*
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Majority of ependymomas diagnosed in adults occur in the spine.
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Less than 6% of CNS tumors in children and adolescents are ependymoma, approximately 230 children are diagnosed with ependymoma per year in the US (ages 0-19).*
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Majority of children diagnosed with ependymoma occur in the brain.
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Ependymomas can spread within the central nervous system (CNS) through the cerebrospinal fluid (CSF). Ependymomas rarely spread outside the CNS.
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Surgery is the main treatment for ependymoma. Additional treatments, such as radiotherapy, chemo and targeted therapy, and clinical trials may be recommended.
Grading of Ependymoma
Ependymomas are grouped in three grades (grade 1, 2, or 3, also written as grade I, II, or III) based on their characteristics under a microscope and their behavior:
Grade 1 ependymomas are low-grade tumors. Subependymomas, an ependymoma subtype, are grade 1 ependymomas that can arise in the brain or the spine. Both are more common in adults than children.
Grade 2 ependymomas are also low-grade tumors. However, they are more likely to recur than grade 1 ependymomas, especially if they cannot be completely removed during surgery. These include the subtypes myxopapillary ependymoma and conventional ependymoma grade 2. Myxopapillary ependymoma typically arises in the spine and is more common in adults. Conventional grade 2 ependymoma can occur in either the brain or spine and are more frequent in adults or children depending on the molecular subtype.
Grade 3 ependymomas are malignant (cancerous). This means they are fast-growing tumors. They occur most often in the brain but can also occur in the spine.
- Ependymoma may spread throughout the CNS although spread outside of the CNS is rare.
- Ependymoma can recur (regrow). Ependymoma recurrences can occur locally (site of the original tumor) or distantly (other locations in the CNS).
- 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.
- Review the Ependymoma Guide for updated information on grading
- 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. These symptoms may be caused by problems other than an ependymoma and should be brought to your doctor’s attention as soon as possible.
Common brain tumor symptoms include:
- Headache or pressure in the head
- Nausea and vomiting
- Blurred vision
- Weakness or numbness and tingling
- Seizures
- Loss of balance or trouble walking
Common spinal cord symptoms include:
- Neck or back pain
- Weakness in the arms or legs
- Numbness or tingling in the arms, legs or trunk
- Problems going to the bathroom, changes in bowel or bladder function
Causes and treatment
- The cause of ependymomas is not known. However, they can occur in people with certain genetic diseases, such as Neurofibromatosis type 2.
- The first treatment for an ependymoma is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and remove as much tumor as possible without causing more symptoms.
- For people that need additional treatment after surgery, treatments may include radiation, chemotherapy or clinical trials. Clinical trials test new chemotherapy, targeted therapy, or immunotherapy drugs. Treatments are decided by the patient’s health care team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.
*Source: Quinn T Ostrom, Mackenzie Price, Corey Neff, Gino Cioffi, Kristin A Waite, Carol Kruchko, Jill S Barnholtz-Sloan, CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2016—2020, Neuro-Oncology, Volume 25, Issue Supplement_4, October 2023, Pages iv1–iv99, https://doi.org/10.1093/neuonc/noad149