What Is the Survival Rate for Ependymoma? Tumor Grades

An ependymoma is a type of primary central nervous system (CNS) tumor that begins in the brain or spinal cord. Ependymoma develops from ependymal cells (radial glial cells), which are one of the three types of glial cells that support the CNS.

Although ependymomas can occur anywhere in the CNS, they often occur near the ventricles in the brain and the central canal of the spinal cord. Rarely, they can occur outside the CNS, such as in the ovaries. 

The ventricles and central canal are spaces where the cerebrospinal fluid (CSF) of the CNS flows. CSF provides nutrition, removes waste material from the CNS, and acts as a shock absorber that prevents physical trauma to the brain and spinal cord.

Ependymoma spreads to other parts of the brain and spinal cord via the CSF, but in rare cases can spread outside the CNS.

Ependymomas of the brain (lower half) are more common in children while ependymomas of the spine are more common in adults. Males are more often affected than females.

Ependymomas are categorized into three grades based on their microscopic characteristics. They are further grouped into subtypes after molecular testing. Molecular testing helps identify the location and disease characteristics of the ependymoma subtypes.

• Grade I ependymoma: Low-grade, slow-growing tumors. Subtypes include subependymoma and myxopapillary ependymoma. Both are more common in adults than in children.
• Grade II ependymoma: Low-grade tumors that can occur in either the brain or the spine.
• Grade III ependymoma: Cancerous, fast-growing tumors. Subtypes include anaplastic ependymomas.

An ependymoma is diagnosed noninvasively via magnetic resonance imaging of the brain and spine. It usually appears as a well-defined mass near the ventricle of the brain or the central canal of the spine. The tumor mass often brightens with contrast.

To accurately diagnose ependymoma and its type, a part of the tumor removed during surgery is examined under a microscope by a neuropathologist.

Symptoms of ependymoma depend on the location of the tumor. Cancerous types can cause multiple symptoms due to the involvement of many areas of the central nervous system.

Symptoms commonly seen with ependymoma in the brain include:

• Headache
• Dizziness
• Nausea
• Vomiting
• Visual disturbances

Symptoms commonly seen with ependymoma in the spine include:

• Backache
• Upper and lower limb weakness
• Problems with urination and bowel evacuation
• Problems in sexual activity

Treatment of ependymoma is planned based on the patient’s age, tumor type, tumor location, coexisting conditions, and remaining tumor after surgery.

Unless the tumor has metastasized to multiple areas in the central nervous system, the primary treatment of ependymoma is surgical removal of the tumor.

There is no standard treatment protocol for ependymomas after surgery. Many people may not require additional treatment if there are little to no tumor tissue remnants after surgery. Other people may need treatment after surgery that may include radiation, chemotherapy, or clinical trials with new chemotherapy, targeted therapy, or immunotherapy drugs.