Living With an Ependymoma

Ependymoma is a rare type of tumor that develops in the lining of the ventricles in the brain and central canal of the spinal cord. Although it can occur at any age, it is more common in young children.

According to data from the Collaborative Ependymoma Research Network (CERN), ependymoma has an overall 5-year relative survival rate of approximately 75% for children below 19 years, 83% for adults aged between 20 and 44 years, and 58% for seniors aged 75 and older. This rate is significantly higher than the 34% average survival rate for patients with all other forms of primary brain and central nervous system tumors.

Living with ependymoma can be challenging, and patients may experience a range of symptoms depending on the location and size of the tumor. This article focuses on what patients need to know about living with ependymoma, its symptoms, diagnosis, treatment, and risk factors.

Some patients with ependymoma may not experience any symptoms,  especially in the early stages of the disease. As the tumor grows, symptoms may begin to appear depending on the type of ependymoma and the location of the tumor.

When the tumor is in the brain, you may experience:

• Feeling nauseous and throwing up
• Persistent or severe headaches
• Seizures
• Loss of balance
• Blurry vision
• Confusion, irritability, fatigue

When the tumor is in the spine, you may experience:

• Problems with sexual performance
• Numbness and weakness in the legs and arms
• Difficulty with urinating and bowel movements
• Stiffness or pain in the neck or back

In children, one of the earliest signs of ependymoma is a condition called hydrocephalus, which occurs when there is a buildup of cerebrospinal fluid in the brain. This can cause the head to grow larger than normal. Children with hydrocephalus may also experience symptoms such as constant crying, sleeplessness, and vomiting. It is important for parents and caregivers to seek medical attention if they notice any changes in their child’s head size or behavior.

Treatment options for ependymoma depend on various factors and can be decided in consultation with a team of medical professionals, including oncologists, neurologists, and neurosurgeons. The treatment plan may involve observing symptoms and monitoring tumor growth through imaging studies like MRI and cerebrospinal fluid tests via spinal taps. The treatment options may include surgery to remove larger tumors, chemotherapy, or radiation therapy to kill the tumor cells. Targeted drug therapy medications may also be used to directly attack the cancer cells.

The prognosis for patients with ependymoma depends on several factors, including age, tumor location, and the effectiveness of treatment. Most patients with ependymoma can expect to live for 5 years or longer. However, infants, children under the age of 19, and adults with tumors outside the spine tend to have worse outcomes. Patients with tumors under the tentorium cerebelli also have a poor prognosis, whereas those with tumors in the spine tend to have better outcomes. It is important for patients to work closely with their medical team to develop a treatment plan that is tailored to their individual needs and circumstances.

To effectively manage ependymoma, patients need to take an active role in their healthcare management. It is important to learn about the condition and what to expect during and after treatment. Maintaining a healthy lifestyle by eating a nutritious diet and engaging in regular exercise can help to improve overall well-being, boost energy levels, and support recovery during and after ependymoma treatment.

Other helpful measures to manage symptoms include staying hydrated, getting enough sleep, and maintaining a positive attitude. Patients with ependymoma should also follow their doctor’s care and symptom management instructions to prevent the recurrence of the tumors.

Although some patients recover completely from ependymoma with proper and early treatment, those with Grade 3 or the historically termed "anaplastic ependymoma" may experience tumor recurrence after treatment. Symptoms of recurrent ependymoma may be subtle at first but can gradually become more severe as the tumors grow. The type of symptoms that you encounter during a recurrence may be like what you experienced before your initial diagnosis. Therefore, patients should stay vigilant and report any changes in symptoms to their healthcare provider.

Survival rates can be worrisome. It is important to note that although statistics can provide valuable information about a population, it cannot predict your individual future. Higher grade tumors such as Grade 3 or anaplastic ependymoma are associated with shorter survival times. However, the specific overall survival time can be difficult to predict because it can vary widely depending on age, tumor subtype, and treatment.

According to a study performed at the M.D. Anderson Cancer Center, adult patients with anaplastic ependymoma lived for about 5 to 6 years after diagnosis. Another study at the Preston Robert Tisch Brain Tumor Center at Duke found that approximately 40% of patients with Grade 3 ependymomas survived at least 10 years.  

Ongoing follow-up care is essential for children with ependymoma and includes regular laboratory tests and imaging to monitor disease progression and any potential medical problems associated with the tumor. The follow-up care schedule will depend on the type of tumor, the patient’s individual needs, and their response to treatment. Because of the high rate of recurrence in children with ependymoma, follow-up care is critical for improving overall quality of life and increasing survival milestones.

Surgery, radiation therapy, and chemotherapy may cause side effects that parents and caregivers will need to manage. Patients may experience confusion, fatigue, muscle weakness, dizziness, and stomach upset, which can last for a prolonged period. However, most of these side effects tend to subside over time after treatment.

Because ependymoma prognosis is specific to each patient, it is essential for parents and guardians to seek advice from medical professionals on the best ways to care for their child. Children diagnosed with this condition require care that prioritizes their comfort, improves their chances of recovery, and reduces the likelihood of relapse after treatment.

• Ependymoma is a type of brain and spinal cord tumor that can cause a variety of symptoms depending on its size and location, including headaches, seizures, loss of balance, blurry vision, numbness, and difficulty with urinating and bowel movements.
• Treatment options for ependymoma depend on various factors and can include surgery, chemotherapy, radiation therapy, and targeted drug therapy medications.
• The prognosis for patients with ependymoma depends on several factors, including age, tumor location, and the effectiveness of treatment.
• Ongoing follow-up care is essential for children with ependymoma and includes regular laboratory tests and imaging to monitor disease progression and any potential medical problems associated with the tumor.

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