Behind the Tumor: Ependymoma

Types

Healthcare providers rate ependymomas on a 1 to 3 grading scale, with grade 1 tumors growing the slowest and grade 3 tumors growing the fastest. There are several types of ependymomas, including:

• Subependymoma (grade 1) grows near the chambers (ventricles) that contain cerebrospinal fluid. It’s more common in adults than children.
• Myxopapillary ependymoma (grade 1) grows in your lower spinal cord and is most common in adult men.
• Classic ependymoma (grade 2) is the most common ependymoma and occurs in children and adults.
• Anaplastic ependymoma (grade 3) often grows near the base of your brain and spreads to other parts of your brain. These tumors tend to return (recur) after treatment.

Location

In children, most ependymomas occur near, or in, the cerebellum and spinal cord. In adults, ependymomas are more commonly found in the spine. Ependymomas are usually located along, within, or next to the ventricular system (spaces in the brain filled with cerebrospinal fluid).

The various types of ependymomas appear in different locations. Subependymomas usually appear near a ventricle. Myxopapillary ependymomas tend to occur in the lower part of the spinal column. Ependymomas are most commonly found in the spinal column in adults and in the lower back part of the skull (posterior fossa) in children.

Population

Ependymomas occur in both children and adults. Those in the lower half of the brain (“posterior fossa”) are more common among children. Those in the spine are more common among adults. Ependymomas occur more often in males than females. They are most common in non-Hispanic white people. An estimated 19,340 people are living with this tumor in the United States. 

Symptoms

Symptoms related to an ependymoma depend on the tumor’s location. Here are some possible symptoms that can occur.  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 
• Sexual, urinary, or bowel problems 

Treatment

Common treatment options for ependymoma include surgery, radiation therapy, and in certain  situations chemotherapy. However, different subtypes may be treated differently. Many patients won’t need additional treatment beyond surgery, depending on the tumor subtype provided that the whole tumor is removed. For example, a subependymoma can be cured by surgery alone and does not typically recur if it is removed completely. If a patient with ependymoma needs more treatment after surgery, it would be either radiation therapy or chemotherapy. However, the role of chemotherapy remains unproven and is still being studied. For now, chemotherapy is often limited to the treatment of very young children and some adults with a recurrent tumor.

The Collaborative Ependymoma Research Network (CERN) was developed to translate lab findings into clinical trials looking at systemic treatment options.

Questions To Ask Your Doctor

It may be helpful to ask your healthcare team the following questions:

• What tests are needed to diagnose ependymoma?
• Does my child or do I need tests to check for neurofibromatosis?
• What are the treatments for ependymoma?
• What are the chances that an ependymoma will return after treatment?

Additional Common Questions

What is the 10-year survival rate for ependymomas?Ependymoma survival rates are more favorable in adults than children. The 10-year survival rate is approximately 70% to 89% in adults. In children, it’s about 64%.