Recovery Outlook for Ependymoma

Ependymoma is a type of tumor that arises from ependymal cells lining the passageways of the brain and spinal cord. Ependymal cells are a type of support cell (glial cell) that play a critical role in the production of cerebrospinal fluid to surround and protect the central nervous system, provide nutrients, and clear wastes.

Most ependymomas are tumors that grow slowly. Although the actual cause of ependymomas is unknown, treatment options such as surgery, radiation therapy, and chemotherapy exist to manage this condition. Survival rates can vary widely depending on tumor, treatment, and patient factors such as tumor grade, extent of tumor removal, and patient age.

Receiving a diagnosis of ependymoma can be a frightening experience, as it often comes with a great deal of uncertainty about the future. Thinking about the implications of a tumor diagnosis on both short-term and long-term plans can be overwhelming. In this article, we will explore the recovery outlook for individuals with ependymoma and its impact on survival.

Ependymomas can grow at different rates. One way to gain a better understanding of how the tumor will progress is by its grade at diagnosis. Tumor grade is a measurement of how abnormal the tumor cells appear when viewed under a microscope and can serve as a valuable tool to predict the aggressiveness of the tumor. Grade 1 ependymomas tend to grow slowly, whereas Grade 3 ependymomas can grow more rapidly. Grade 2 ependymomas have an intermediate growth rate that is generally less predictable than Grade 1 or Grade 3 tumors.

Symptoms can be subtle and occur infrequently during the early stages of the tumor. However, as the tumor grows, symptoms can intensify, persist, and cause significant impairment. The exact symptoms will vary based on the location of the tumor and the patient’s age. Adults may mention symptoms such as nausea, headaches, back pain, weakness, numbness, and vision changes, whereas young children may become more irritable, have difficulty sleeping, or exhibit abnormal head growth.

Prompt diagnosis and treatment will help to prevent further growth and potential spread. Without treatment, the tumor may grow and worsen symptoms. Although surgery, radiation therapy, and sometimes chemotherapy are used to manage this condition, some patients may opt for a “wait-and-see” approach. This may be particularly favorable for older patients with slow-growing Grade 1 ependymomas that are not causing symptoms.

Understanding the impact of an ependymoma diagnosis on survival can be a challenging and frustrating process. Although some patients diagnosed with ependymoma as children go on to lead long and productive lives more than 50 years after treatment, others may succumb to the disease much earlier or experience negative effects from tumor recurrence and multiple treatments.

Ependymoma survival statistics can be helpful to know the general trajectory of disease but are not predictive of your individual future. Here is what we know about ependymoma prognosis based on important factors influencing outcomes:

• Age: Adults have higher survival rates than children. According to the National Cancer Institute, 10-year survival rates for adults and children are approximately 75% and 64%, respectively.
• Location: Spinal ependymomas have higher survival rates and lower recurrence rates than those that occur in the brain. According to a multi-institutional study funded by the Collaborative Ependymoma Research Network Foundation, 5-year survival rates for adult spinal, infratentorial, and supratentorial ependymomas were 97%, 85%, and 62%, respectively.
• Treatment: Complete surgical tumor removal, or gross total resection (GTR), has consistently been associated with higher survival rates and lower recurrence rates than tumors that were incompletely removed. Radiation is beneficial for preventing recurrence particularly for tumors at the lower parts of the brain (infratentorial).
• Grade: Higher grade tumors (Grade 3) have worse survival rates and higher recurrence rates than lower grade tumors (Grade 1). Grade 3 anaplastic ependymomas have 10-year overall survival rates of approximately 50% compared with 80% for other ependymomas on average. The median time to an ependymoma recurrence (progression-free survival) is approximately 2 years for a Grade 3 ependymoma and 16 years for a Grade 2 ependymoma. Grade 1 ependymomas rarely recur, so recurrence data are limited.
• Molecular features: Certain molecular features are associated with worse or better outcomes. Supratentorial ependymomas with YAP1 fusion have better prognoses than those that are ZFTA fusion-positive. Posterior fossa Group B ependymomas have better prognoses than Group A. Most spinal ependymomas are associated with excellent survival rates and outcomes except for the aggressive MYCN-amplified subtype.

Although survival rates are useful, they do not provide direct insight into the risk of recurrence. The treatment journey for ependymoma patients can vary from being a relatively straightforward process, such as a single surgical operation and radiation treatment, to a more prolonged and complex one involving multiple treatments because of tumor recurrence. When multiple treatments are required, patients are exposed to the risks and potential consequences of treatment repeatedly, making the process more challenging.

Therefore, it is critical to consider the risks of treatment and each additional intervention and weigh them against potential benefits. This is especially crucial for young children, as radiation therapy can have long-term effects on cognitive and behavioral development. Regular discussions about treatment goals, potential risks, and benefits should be held with your medical team to ensure that informed decisions are made throughout the treatment process.

Complete surgical removal is a crucial factor in predicting long-term survival for ependymoma patients. Therefore, finding an experienced neurosurgeon who specializes in treating this type of tumor is one of the most critical decisions you can make for your care. Learn more about how to find an expert neurosurgeon for your ependymoma here.

Surgery is typically the primary treatment for ependymomas, with the goal of safely removing as much of the tumor as possible without harming nearby structures. The location of the tumor will determine the location of the incision, which may be on the head or back. Following surgery, patients are taken to a recovery unit and then transferred to the intensive care unit for pain management and close monitoring of vital signs such as heart rate and blood pressure.

Once stable, patients are taken to a hospital room to rest and recover. In the next few days, patients may be encouraged to eat and move out of bed as able. Physical and occupational rehabilitation are often necessary after ependymoma surgery to help patients regain strength, mobility, and learn how to use their bodies within any limitations that may have resulted from the procedure. This can involve exercises, therapies, and other interventions to help patients regain function and independence.

In addition to rehabilitation, patients may need assistance with daily tasks, such as meal preparation, cleaning, and personal care, as they recover at home. Family members, caregivers, or home health aides may provide this support, as needed.

It is common to experience fatigue and pain around the incision following ependymoma surgery, especially within the first few days. However, as time goes on, patients can expect their energy level to increase and their pain to decrease. Continued rehabilitation may be necessary to restore as much function as possible. While it can take up to 6 weeks or longer to feel fully recovered, abilities such as walking or speech may take longer to recover with rehabilitation, depending on the initial severity.

It is important for patients to follow up with their surgeon to discuss next steps and to plan for imaging to monitor for tumor recurrence. Attending these appointments is critical to ensure that the tumor is not growing. There are numerous inspiring stories from patients and caretakers of patients who have made commendable recoveries after ependymoma treatment, and with proper care and support, many patients can achieve significant improvements in their health and well-being.

• Ependymoma 10-year survival rates vary widely from 60% to over 90%.
• Lower tumor grade and complete surgical resection are predictors of long-term survival and lower recurrence rates.
• Spinal ependymomas have more favorable prognoses when compared with those located in the brain.
• Recovery after ependymoma surgery can take up to 6 weeks or longer.
• Physical and occupational rehabilitation are often used after ependymoma surgery to maximize recovery and functional independence.

• Collaborative Ependymoma Research Network (CERN)
• American Brain Tumor Association (ABTA): Ependymoma
• The Childhood Brain Tumor Foundation 
• Pediatric Brain Tumor Foundation