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Ependymoma Diagnosis and Treatment

MRI of an ependymoma in the spine.

Credit: NCI-CONNECT Staff

An ependymoma is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of ependymomas?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Ependymomas are grouped in three grades based on their characteristics (grade 1, 2, or 3, also written as grade I, II, or III). Within each grade, are different ependymoma subtypes. Molecular testing is used to help identify subtypes that are related to location and disease characteristics.

  1. Grade 1 ependymomas are low grade tumors. This means the tumor cells grow slowly. The subtypes include subependymoma and myxopapillary ependymoma. Both are more common in adults than in children. Myxopapillary tumors usually occur in the spine.
  2. Grade 2 ependymomas are low grade tumors and can occur in either the brain or the spine.
  3. Grade 3 ependymomas are malignant (cancerous). This means they are fast-growing tumors.  The subtypes include anaplastic ependymomas.  These most often occur in the brain, but can also occur in the spine.

What do ependymomas look like on an MRI?

Ependymomas usually appear as a well-defined mass that often brightens with contrast. 

What causes ependymomas?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. The cause of ependymomas is not known.

Where do ependymomas form?

Ependymomas can form anywhere in the CNS. Ependymomas often occur near the ventricles in the brain and the central canal of the spinal cord. On rare occasions, ependymomas can form outside the CNS, such as in the ovaries.  Ependymomas develop from ependymal cells (called radial glial cells). Ependymal cells are one of three types of glial cells that support the CNS.

Do ependymomas spread?

Ependymomas rarely spread outside the CNS. But ependymomas can spread to other areas of the CNS through cerebrospinal fluid (CSF).

What are symptoms of an ependymoma?

Symptoms related to an ependymoma depend on the tumor’s location. Here are some possible symptoms that can occur.

Ependymoma Symptoms

People with an ependymoma in the brain may have:

  • Headaches
  • Nausea
  • Vomiting
  • Dizziness

People with an ependymoma in the spine may have:

  • Back pain
  • Numbness and weakness in their arms, legs or trunk
  • Problems with sexual, and urinary or bowel problems

Who is diagnosed with ependymomas?

Ependymomas occur in both children and adults.  Ependymomas in the lower half of the brain are more common among children. Ependymomas in the spine are more common among adults. Ependymomas occur more often in males than females. They are most common in white and non-hispanic people. An estimated 13,294 people are living with this tumor in the United States.

Ependymoma Statistics

What is the prognosis of ependymomas?

The likely outcome of the disease or chance of recovery is called prognosis.

Ependymoma Prognosis

The relative 5-year survival rate for ependymoma is 83.9% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are treatment options for ependymomas?

The first treatment for an ependymoma is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person. 

After surgery, there is no standard treatment for ependymomas. Many people won’t need other treatment after surgery. For other people, treatments may include radiation, chemotherapy or clinical trials. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.

Open Clinical Studies for Ependymomas

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