What Is Ependymoma?

Overview

Within your brain and spinal cord, there are spaces known as ventricles that contain cerebrospinal fluid. This fluid plays a crucial role in protecting the brain and spinal cord against injuries and shielding them from infections or toxins. 

The lining of these ventricles consists of specialised cells, known as ependymal cells, which produce cerebrospinal fluid. An ependymoma is an uncommon tumour that originates from an overgrowth of these cells. Ependymomas are considered rare and can occur in various locations within the brain and spinal cord where these ventricles are present.

This article will explore the signs and symptoms, causes and management options for ependymomas. 

Types of ependymoma

Depending on where it is located and how aggressive it is, an ependymoma can be categorised into four primary types:¹

• Subependymoma (grade 1): These tumours grow slowly in ventricles and are often benign (non-cancerous) 
• Myxopapillary ependymoma (grade 1): These tumours are more commonly found in adults than in children. They are typically localised to the base of the spine
• Classic ependymoma (grade 2): These tumours are well-defined and typically occur in the lower region of the brain, which includes the cerebellum and brain stem. They can also appear in the spinal cord or the upper part of the brain known as the cerebral cortex
• Anaplastic ependymoma (grade 3): These tumours tend to grow rapidly and often indicate a poor prognosis (outcome). They usually develop in the posterior fossa, which is the area of the brain that encompasses the cerebellum and brain stem, before spreading to other parts of the brain

Tumour grades are a measure of how quickly they grow, with grades 1 and 2 being slower growing and grade 3 being faster growing.

Causes of ependymoma

Similar to many brain tumours, the exact causes of ependymomas are unknown. However, researchers have observed a tendency for ependymomas to occur more frequently within families, suggesting a potential inherited risk. 

Additionally, individuals with neurofibromatosis type 2 (NF2), a condition characterised by the growth of non-cancerous tumours in the nervous system, have a higher likelihood of developing spinal ependymomas. 

Signs and symptoms of ependymoma

One of the primary signs of ependymomas is the presence of brain pressure, which often leads to headaches. These headaches are commonly experienced upon awakening in the morning. 

In children, ependymomas may also result in hydrocephalus, characterised by an accumulation of fluid in the brain. This can lead to an increase in head size, particularly in infants. 

Additional symptoms associated with ependymomas include:

• Nausea and vomiting, which tend to occur more frequently in the morning
• Fatigue and a general lack of energy
• Irritability
• Difficulties with walking, as ependymomas can affect balance and cause the eyes to cross

In children, approximately 90% of ependymomas are located in the brain, while the majority of cases in adults are found in the spine. Spinal tumours can manifest as backaches, soreness, and tingling sensations in the legs. 

Management and treatment for ependymoma

The primary approach to treating an ependymoma is typically neurosurgery, with the aim of removing as much of the tumour as possible.^2,3 However, in certain cases, the tumour's location may pose challenges that make complete removal unfeasible. 

When hydrocephalus is present, surgeons may opt to insert a shunt (a thin tube) into the brain to help drain excessive cerebrospinal fluid. This involves placing a tube that carries the fluid from the brain, typically redirecting it to the abdomen where it is safely absorbed by the body. 

In situations where surgery alone cannot completely eliminate the tumour, radiation therapy may be employed to help reduce its size. This involves using radiation beams that specifically target the tumour while minimising damage to surrounding healthy tissues. 

In some cases, chemotherapy medications may be administered prior to surgery to shrink the tumour.

If the cancer has metastasised (spread to other parts of the body), radiation therapy or chemotherapy may be considered as the primary treatment options.^1,3 

The choice of treatment depends on various factors, including the individual's age, the tumour's location, and the overall health of the patient. 

Diagnosis

To diagnose an ependymoma, multiple tests may be necessary. The initial step involves a doctor evaluating your symptoms and medical history. A physical examination will be conducted, which includes assessing reflexes, coordination, eye and facial movement, as well as muscle strength. 

Additional diagnostic tests can include:

• Magnetic resonance imaging (MRI): This noninvasive procedure employs a magnetic field and radio waves to generate detailed images of the brain or other targeted organs. It does not involve radiation exposure 
• Computed tomography (CT) scan: A specialised type of X-ray that produces cross-sectional images, allowing for more precise visualisation of the brain compared to standard X-rays 
• Biopsy: This invasive test involves the surgical removal of a small tissue sample. It may also be obtained with a needle, depending on the tumour's location. The extracted tissue is then examined in a laboratory to determine if it is cancerous 
• Lumbar puncture (spinal tap): Another invasive procedure, in which a needle is used to withdraw a sample of cerebrospinal fluid from the spinal cord. The collected sample is examined to detect the presence of cancer cells moving through the nervous system 

FAQs

How can I prevent ependymomas?

Currently, there are no known specific methods for preventing ependymomas. Since the causes of ependymoma are not well understood, it is challenging to identify preventive measures. 

However, there are general lifestyle choices that may contribute to overall brain health and potentially reduce the risk of developing brain tumours, including ependymomas: 

• Maintaining a healthy lifestyle 
• Protect yourself from radiation
• Protect yourself from environmental toxins 

How common is ependymoma?

Ependymomas primarily affect children who are under the age of 5. Boys are more commonly affected by this type of tumour compared to girls. According to Cancer Research UK, approximately 30 children in the UK receive a diagnosis of ependymoma each year. 

Who is at risk of ependymoma?

Ependymomas are predominantly found in children, particularly infants and young children, rather than in adults. This means that paediatric cases are generally more common, putting children at a higher risk.

When should I see a doctor?

If you suspect that you may have an ependymoma or are experiencing symptoms that concern you, it is important to seek medical attention promptly. 

• Persistent or worsening symptoms: If you experience persistent or worsening symptoms that are concerning, such as persistent headaches, nausea, vomiting, unexplained weight loss, changes in vision, difficulty with coordination or balance, or any other neurological symptoms
• New or unexplained symptoms: If you develop new or unexplained symptoms that persist for an extended period, such as changes in motor skills, seizures, speech difficulties, or unexplained pain
• Family history or genetic predisposition: If you have a family history of ependymoma or other brain tumours, or if you have a known genetic predisposition to such conditions

If you experience any of the above, it is recommended you speak to your GP or doctor. 

Summary

Ependymomas are uncommon tumours that develop from the cells lining the ventricles in the brain and spinal cord. They can occur in various locations and are categorised into four types based on their location and aggressiveness: subependymoma, myxopapillary ependymoma, classic ependymoma, and anaplastic ependymoma. 

The exact causes of ependymomas are unknown, but there is a potential inherited risk and a higher likelihood for individuals with neurofibromatosis type 2. Ependymomas primarily affect children under the age of 5, with boys being more commonly affected. 

Symptoms of ependymomas include headaches, hydrocephalus, nausea, vomiting, fatigue, irritability, and difficulties with walking. Treatment options involve neurosurgery, radiation therapy, and chemotherapy. Diagnosis involves evaluations of symptoms, physical examination, and diagnostic tests such as MRI, CT scan, biopsy, and lumbar puncture. 

There are no specific methods for preventing ependymomas, but maintaining a healthy lifestyle and minimising radiation and environmental toxin exposure may contribute to reducing the risk. 

It is important to seek medical attention promptly if you are experiencing persistent or worsening symptoms, new or unexplained symptoms, or if you have a family history of brain tumours.