Overview
Ependymoma is a rare tumor of the brain or the spinal cord that starts in cells of the ependyma, which is a thin lining of cells of the ventricular system of the brain and the central canal of the spinal cord. The ventricular system of the brain is a set of four connected cavities that are concerned with the production and circulation of cerebrospinal fluid (CSF). Signs and symptoms largely depend on the tumor’s location, but can include one-sided weakness, difficulty with speech or language, and vision changes. Seizures may occur. Headache, nausea, and vomiting may occur due to increased pressure within the skull. Back pain, changes in movement, and changes in sensation can occur due to changes related to the spinal cord.
Several subtypes of ependymoma are further classified based on the anatomical location of the tumor, the tissue, and the tumor’s biomarkers. Biomarker testing, also called molecular testing, plays an important role in the diagnosis and subtyping of ependymomas. Subtypes of ependymoma include the following:
- Supratentorial ependymoma
- Supratentorial ependymoma, ZFTA fusion-positive
- Supratentorial ependymoma, YAP1 fusion-positive
- Posterior fossa ependymoma
- Posterior fossa group A (PFA) ependymoma
- Posterior fossa group B (PFB) ependymoma
- Spinal ependymoma
- Spinal ependymoma, MYCN amplified
- Myxopapillary ependymoma
- Subependymoma
2021 WHO CNS Grade
A tumor grade describes how normal or abnormal tumor cells appear under a microscope. In lower grade tumors like grade 1, the cells look close to normal. In higher grade tumors like grade 4, the cells look more abnormal. Ependymoma is considered grade 1, 2, or 3.
Statistics
- Percent of All Brain and Other CNS Tumors: 1.5%
- Average Annual Age-Adjusted Incidence Rate: 0.41 per 100,000
- Median Age at Diagnosis: 46 years
Survival
Survival rates compare the percentage of people living with a particular diagnosis at a particular point in time compared to the general population.
- One-Year Relative Survival Rate: 96.2%
- Five-Year Relative Survival Rate: 91.0%
- Ten-Year Relative Survival Rate: 87.2%
Treatment Options
Every person living with a brain or CNS tumor is unique. If you have been diagnosed with a brain or CNS tumor and would like to better understand your treatment options, it is important to work with your health care team to decide the best course of treatment for you. You may also consider getting a second opinion or reaching out to our Personalized Support and Navigation team to learn more.
