Ependymomas arise from the ependymal cells that line the ventricles of the brain and the center of the spinal cord. They are soft, grayish, or red tumors which may contain cysts or mineral calcifications.
Ependymomas are classified by the following types using histopathological, molecular, and anatomic features:
- Subependymomas (grade 1): Typically slow-growing tumors.
- Myxopapillary ependymomas (grade 2): Typically slow-growing tumors.
- Ependymomas (grade 2-3): The most common of the ependymal tumors.
The following patterns of histopathological description can still be used but no longer classified as subtypes: papillary ependymomas, clear cell ependymomas, and tancytic ependymomas.
The following are ependymoma subtypes that occur in specific locations:
- Supratentorial location
- Supratentorial ependymoma, ZFTAfusion-positive
- Supratentorial ependymoma, YAP1fusion-positive
- Posterior fossa location
- Posterior fossa ependymoma, group PFA
- Posterior fossa ependymoma, group PFB
- Spinal cord
- Spinal ependymoma, presence or absence of MYCN-amplification
- Myxopapillary ependymoma
- Subependymoma
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.
Symptoms
Symptoms of ependymoma are related to the location and size of the tumor. Common symptoms may include headaches; nausea and vomiting; dizziness; vision changes; seizures; personality changes; weakness or numbness and tingling on one side of the body; bowel or bladder changes; and cognitive problems related to thinking, learning, concentrating, problem-solving, and decision making.
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 and 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.
Prognosis
Prognosis means a prediction of outcome. This information is usually based on information gathered from groups of people with the same disease. It is important to remember these statistics are not individualized.
The 5-year relative survival rates for non-malignant ependymoma by age group are as follows:
- Children (0-14): 96.8%
- Adolescents and Young Adults (15-39): 98.7%
- Adults (40+): 95.8%
The 5-year relative survival rates for malignant ependymoma by age group are as follows:
- Children (0-14): 74.7%
- Adolescents and Young Adults (15-39): 91.7%
- Adults (40+): 86.4%
Incidence
Ependymomas are relatively rare, accounting for less than 2% of all brain and other CNS tumors. They account for about 9% of all childhood central nervous system (CNS) tumors, and about 2% of adult CNS tumors. Ependymomas occur slightly more in males than females. They occur more commonly in white and non-Hispanic people. Grade 3 ependymomas are more common in adults.
Age DIstribution
The median age of ependymoma diagnosis in children is 5 years old and in adults is 30-40 years old.
Risk Factors
The exact cause of most ependymomas is unknown. One known risk factor for developing ependymoma is an inherited cancer syndrome called neurofibromatosis type 2. However, people who inherit the genetic changes that cause neurofibromatosis type 2 do not inherit ependymoma, meaning that the risk of developing the tumor is not passed down to family members. Only a small number of patients have this cancer syndrome that increases their chance of developing ependymoma and not all patients with this syndrome develop ependymoma.
Molecular Profile
Molecular profiling is the detection of specific genes, proteins, or other molecules in a tumor. This information helps confirm tumor diagnosis, inform treatment options, and predict prognosis.
Genetic and Molecular markers commonly altered with ependymoma tumors include the following:
- Supratentorial Ependymomas: ZFTA, RELA, YAP1, MAML2
- Posterior Fossa Ependymomas: H3 K27me3, EZHIP (methylome)
- Spinal Cord Ependymomas: NF2, MYCN
Content last reviewed:
November 2022 by Ashley Ghiaseddin, MD
