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Ependymoma is a tumor of the brain and spinal cord. These tumors are more common in children, particularly infants and young children. However, they may also occur in adults. There are about 200 new cases of ependymoma every year in the United States in children and young adults under 21 years of age. Ependymoma is the 3rd most common brain tumor in children.
Ependymomas are thought to arise from cells that develop into ependymal cells that line the fluid-filled ventricles of the brain and central canal of the spinal cord. These tumors can sometimes spread through the cerebrospinal fluid (CSF).
In children, approximately 70%-80% of ependymomas occur in the posterior fossa, but they can develop in different regions of the central nervous system (CNS). Depending on the location of the tumor, different parts of the CNS may be affected including the:
Many ependymomas form in the fourth ventricle in the posterior fossa. Tumors in this location are most likely to affect the brain stem and cerebellum.
Treatment for ependymoma includes surgery to remove as much of the tumor as possible. Radiation therapy is often used along with surgery to kill any remaining cancer cells. Chemotherapy may also be used before or after surgery.
The survival rate for childhood ependymoma is 50-70%, depending upon the location of the tumor, the extent of resection and the molecular subgroup.
Symptoms of childhood ependymoma vary and depend on several factors including:
Ependymoma symptoms may include:
Doctors evaluate ependymoma in several ways.
Ependymoma tumors are identified by how they look under the microscope. The more abnormal the cells look, the higher the grade. High grade tumors are more aggressive and can spread throughout the brain and spinal cord.
Certain types of ependymoma are associated with a better outcome than other types. However, other factors also affect treatment and prognosis.
|Tumor Subtypes Based on Histology
||Outcomes in General
|Grade I||Low Grade Tumor: Localized, grow slowly||Subependymoma
|Grade II||Cellular ependymoma
Clear cell ependymoma
|Grade III||High Grade Tumors: Grow quickly and spread to other places; High rate of recurrence
|| Anaplastic ependymoma
The prognosis for ependymoma depends on multiple factors. Children with certain types of ependymoma tend to have a better chance for long-term survival. However, late recurrences or relapse are not uncommon in ependymoma. Approximately 50% of children have a relapse of their disease within 10 years from diagnosis.
Factors that influence chance of cure include:
Scientists are learning more about the molecular aspects of ependymoma. These advances can help doctors better understand the disease and plan treatments. Certain tumor features, like the presence of C11orf95-RELA fusion in supratentorial tumors and extra copies of the chromosome 1q in posterior fossa tumors, have been linked to increased chance of relapse.
Ependymoma is difficult to cure without successful surgical removal of the tumor. When ependymoma recurs, the long-term prognosis is usually poor.
Treatment of ependymoma depends on the type and location of tumor, age of the child, and whether the tumor is newly diagnosed or recurrent.
Surgery to remove as much of the tumor as possible is the main treatment for ependymoma. The goal is gross total resection, or complete removal of the tumor. For patients with some grade I tumors, surgery alone may be an effective treatment depending on tumor type, location and the extent of resection.
Radiation therapy is usually used after surgery for ependymoma. The type of radiation therapy used depends on the type and location of the tumor.
Chemotherapy is often used along with focal radiation therapy and surgery. Chemotherapy may be used before surgery to make surgical removal of the tumor easier. In very young children, chemotherapy may be used to help delay radiation until the child is older.
Children who have a recurrence of disease following initial surgery and radiation therapy may be offered treatment within a clinical trial. There is a lack of successful standard therapies for recurrent ependymoma. Clinical trials include the use of immunotherapy drugs targeting the immune system to help fight the cancer and biologic agents or antibodies acting on specific proteins in the tumor cells.
Periodic imaging is used to monitor patients long-term with the surveillance schedule based on type of tumor and response to treatment. Follow-up care should include appropriate rehabilitation, neurological, and endocrinology consultations to monitor for adverse effects of treatment. Assessment of neurocognitive function, vision, and hearing is also important. Incorporating palliative care and support services such as psychology and social work can help patients and families manage symptoms, promote quality of life, and navigate care decisions.
Reviewed: June 2018