Ependymomas are rare primary brain and spinal cord tumors that originate from ependymal cells lining the ventricles of the brain and the central canal of the spinal cord. They can occur at any age but are more common in children and young adults. Ependymomas are classified based on their location, histological features, and genetic markers, which can influence their behavior and treatment approach.

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Classification and Grades

Ependymomas are classified into several types and grades according to the World Health Organization (WHO):

• Grade I: Myxopapillary ependymomas and subependymomas, typically slow-growing and considered less aggressive. Myxopapillary ependymomas usually occur in the spinal cord, while subependymomas are often found in the fourth ventricle of the brain.

• Grade II: Classic ependymomas, which include cellular, papillary, and clear cell ependymomas. These are slow to moderately growing tumors that can occur anywhere in the central nervous system but are more common in the brain in children and in the spinal cord in adults.

• Grade III: Anaplastic ependymomas, characterized by high cellularity, mitotic activity, and often more aggressive behavior. They have a higher potential to recur and spread within the central nervous system compared to lower-grade ependymomas.
   

Symptoms

The symptoms of ependymomas depend on the tumor's location and size and may include headaches, nausea, vomiting, seizures, weakness, or numbness in parts of the body, balance and coordination problems, and changes in vision or hearing.

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Diagnosis

Diagnosis involves a neurological exam followed by imaging tests, such as MRI or CT scans, to locate and characterize the tumor. A biopsy or surgical resection may be required to confirm the diagnosis through histological examination.

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Treatment

Treatment options for ependymomas may include:

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• Surgery: The primary treatment for ependymomas is surgical removal, with the goal of removing as much of the tumor as possible while preserving neurological function.

• Radiation Therapy: Often used after surgery, especially for higher-grade tumors or when complete surgical removal isn't possible, to kill remaining cancer cells and reduce the risk of recurrence.

• Chemotherapy: Less commonly used for ependymomas, but may be considered in certain cases, such as for young children or for tumors that have recurred or spread.
   

Survival Rates and Prognosis

The prognosis for ependymoma patients varies significantly based on the tumor's grade, location, the extent of surgical removal, age at diagnosis, and other factors. Overall, the 5-year survival rate for ependymoma patients is around 60-70%, but this figure can vary widely:

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By Grade

• Grade I Ependymomas (Myxopapillary Ependymomas and Subependymomas): These tumors generally have the best prognosis, with 5-year survival rates exceeding 90% in many cases, especially when complete surgical resection is possible. Long-term survival is also favorable, with rates still high at 10 years post-diagnosis.

• Grade II Ependymomas: The 5-year survival rate for patients with Grade II ependymomas can vary but generally ranges from 70% to 80%. The prognosis can be better for those who undergo complete surgical removal and receive postoperative radiation therapy if needed.

• Grade III Ependymomas (Anaplastic Ependymomas): These are the most aggressive ependymomas with a poorer prognosis. The 5-year survival rate ranges from 50% to 60%, with survival rates decreasing further for 10-year survival. Anaplastic ependymomas are more likely to recur and may require additional treatments.
   

By Age

• Children: The prognosis for children, especially those younger than 3 years, can be less favorable due to the challenges in administering radiation therapy and the aggressive nature of some pediatric ependymomas. However, advances in pediatric cancer treatment have improved outcomes, with overall 5-year survival rates for pediatric ependymoma patients ranging from 60% to 70%.

• Adults: Adults with ependymoma generally have better survival rates compared to children, especially when tumors are located in the spinal cord. Adult 5-year survival rates can vary widely based on tumor grade and location but tend to be higher on average than those for children.
   

By Location

• Spinal Cord Ependymomas: These tend to have a better prognosis than their intracranial counterparts, partly because they are more likely to be completely resected and are often lower grade. The 5-year survival rate for spinal ependymomas is generally high, especially for Grade I tumors.

• Intracranial Ependymomas: Survival rates for ependymomas in the brain vary more significantly based on the specific location within the brain, the tumor's grade, and the patient's age. Complete resection is more challenging for intracranial ependymomas, which can affect survival rates.
   

Recurrence

Recurrence significantly affects survival rates and prognosis. Patients with ependymoma, especially those with higher-grade tumors, require careful long-term monitoring for recurrence, which can impact survival rates and overall outcomes.

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It's crucial for patients and their families to discuss their specific case with their healthcare team to understand the prognosis and survival rates in the context of their unique situation. The ongoing research into the molecular and genetic underpinnings of ependymomas holds promise for developing more targeted and effective treatments, potentially improving future survival rates.

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Ongoing Research

Research efforts are ongoing to better understand the molecular and genetic characteristics of ependymomas, which could lead to more personalized treatment approaches and improved outcomes. Clinical trials are exploring new therapeutic agents, including targeted therapies and immunotherapies, offering hope for patients with these challenging tumors.

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