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Ependymoma

What is Ependymoma?

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).

Many ependymomas form in the fourth ventricle in the posterior fossa region. Tumors in this location are most likely to affect the brain stem and cerebellum.

Many ependymomas form in the fourth ventricle in the posterior fossa region. Tumors in this location are most likely to affect the brain stem and cerebellum.

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:

  • Cerebrum
  • Cerebellum
  • Brainstem
  • Spinal cord

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.

Signs and Symptoms of Ependymoma

Symptoms of childhood ependymoma vary and depend on several factors including:

  • Location of the tumor in the brain
  • Size of the tumor
  • Child’s age and stage of development

Ependymoma symptoms may include:

  • Nausea and vomiting
  • Headache
  • Back or neck pain
  • Vision problems
  • Loss of balance or problems walking
  • Leg weakness
  • Seizures
  • Irritability or confusion
  • Problems urinating or change in bowel function

Diagnosis of Ependymoma

Doctors evaluate ependymoma in several ways.  

  • A health history and physical exam provide information about symptoms, general health, past illness, and risk factors.
    • Most ependymomas occur in infants and young children. The cause of ependymoma is unknown. However, there are certain genetic factors that may increase risk including neurofibromatosis.
  • A neurological exam measures different aspects of brain function including memory, vision, hearing, muscle strength, balance, coordination, and reflexes.
  • Imaging tests are used to help identify the tumor. Magnetic resonance imaging (MRI) of the brain and spinal cord is the main imaging technique that is usually used to evaluate ependymoma. MRI uses radio waves and magnets to make detailed pictures of the body. The images made by MRI can give more information about the type of tumor and the potential spread of disease. MRI is also done after surgery to see if any tumor remains.
  • A lumbar puncture may be performed to look for cancer cells in the cerebrospinal fluid.
  • A biopsy is used to diagnose ependymoma. In a biopsy, a small sample of the tumor is removed during surgery. A pathologist looks at the tissue sample under a microscope to identify the specific type and grade of ependymoma.

Grading and Staging of Ependymoma

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
Myxopapillary ependymoma
Favorable
Grade II    Cellular ependymoma
Papillary ependymoma
Clear cell ependymoma
Tanycytic ependymoma
Intermediate 
Grade III    High Grade Tumors: Grow quickly and spread to other places; High rate of recurrence 
 Anaplastic ependymoma
Poor

Prognosis for 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:

  • Location of the tumor
  • Type and grade of the tumor
  • Whether the cancer is localized or has spread to other areas of the brain
  • If surgery can completely remove the tumor
  • Age at diagnosis
  • If the cancer has come back (relapse or recurrence)
  • Molecular features of the tumor

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

Treatment of ependymoma depends on the type and location of tumor, age of the child, and whether the tumor is newly diagnosed or recurrent.

  1. 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.

  2. Radiation therapy is usually used after surgery for ependymoma. The type of radiation therapy used depends on the type and location of the tumor.

  3. 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.

  4. 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.

Life after Ependymoma  

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.   

More: Life After Brain Tumors


Reviewed: June 2018