What is ependymoma?

Ependymoma is a rare type of brain tumour that grows in the brain or spinal cord. It originates from the ependymal cells, which line the ventricles within the brain and the central canal of your spinal cord. These cells produce cerebrospinal fluid, which helps cushion and protect your brain and spinal cord. Ependymomas develop when these ependymal cells multiply uncontrollably, forming a mass or tumour within the central nervous system.

Ependymomas are a rare brain tumour originating in the brain or spinal cord.

There are three main types of ependymoma, which are categorised based on their location and severity. The three types of ependymoma include:

  • Subependymoma (Grade 1): more common in adults and grows near the ventricles in the brain.
  • Myxopapillary ependymoma (Grade 1): more common in male adults and grows in the spinal cord.
  • Classic ependymoma (Grade 2): most common type of ependymoma and grows in the brain of both adults and children.
  • Anaplastic ependymoma (Grade 3): grows faster than the other types of ependymomas and near the base of the brain. This type of ependymoma tends to be more aggressive, can spread to other parts of the brain, and has a greater likelihood of recurrence.

What causes ependymomas?

The cause of ependymomas is still unknown; however, cancer, in general, is caused by genetic mutations which cause cells to mutate and grow out of control. This causes tumours to grow, resulting in cancer. 

What are the symptoms of ependymomas?

Ependymomas can produce a range of symptoms, and the specific signs a person experiences often depend on the tumour's location within the central nervous system. Here are some common symptoms associated with ependymomas:

  • Headaches: persistent and often worsening headaches are a frequent complaint among individuals with ependymoma.
  • Nausea and vomiting: increased intracranial pressure due to the tumour can result in nausea and vomiting, especially in the morning or after physical activity.
  • Seizures: ependymomas near the brain's surface can irritate surrounding brain tissue, potentially triggering seizures. These seizures may present as unusual movements, changes in consciousness, or sensory disturbances.
  • Changes in vision or balance: ependymomas that affect specific areas of the brain responsible for vision or balance can lead to visual disturbances, such as double vision, also known as diplopia or blurry vision, and problems with coordination and balance.
  • Weakness or numbness: ependymomas along the spinal cord can compress or interfere with nerve function, leading to weakness or numbness in the extremities. This can affect your arms, legs, or both.
  • Hydrocephalus: ependymomas within the brain's ventricular system can obstruct the normal circulation of cerebrospinal fluid, leading to a condition called hydrocephalus. Symptoms may include a rapidly enlarging head circumference in infants or signs of increased intracranial pressure in older children and adults.
Hydrocephalus can occur in infants and children with ependymomas.

Is ependymoma painful?

Ependymomas can cause pain and discomfort depending on the grade and location of the tumour. Individuals with spinal ependymoma may experience neck and back pain, while those with intracranial/brain ependymoma may experience headaches.

Who is at risk of ependymomas in Singapore?

Ependymoma is a rare type of brain tumour, and while its exact causes are not fully understood, certain factors may increase the risk of developing this condition, such as:

  • Age: ependymomas can affect individuals of all ages, but they are more commonly diagnosed in children and young adults, with a significant number of cases occurring in those under the age of 5.
  • Genetic predisposition: researchers have identified a significant link between ependymoma development and a genetic disorder known as neurofibromatosis type 2 (NF2). Individuals with neurofibromatosis type 2 have a higher risk of developing various nervous system tumours, including ependymomas.

lumbar puncture
A cerebrospinal fluid analysis will be performed to measure the chemicals present in the CSF.

How is ependymoma diagnosed?

Diagnosing ependymoma involves a comprehensive evaluation and a series of diagnostic tests to gather crucial information about the tumour. Here is an overview of the diagnostic steps:

  • Clinical assessment: initially, your neurosurgeon will conduct a thorough clinical assessment. They will inquire about your symptoms, medical history, and family history of neurological conditions or tumours. During a neurological examination, your doctor will assess your vision, hearing, balance, coordination, and reflexes.
  • Imaging tests: imaging tests, such as magnetic resonance imaging (MRI), are used to diagnose ependymoma. It provides detailed, high-resolution images of your brain and spinal cord, which helps identify the tumour's location, size, and characteristics.
  • Lumbar puncture: a lumbar puncture, also known as a spinal tap or cerebral spinal fluid analysis, may be recommended when diagnosing ependymoma. During this procedure, a needle is carefully inserted between two lower spinal bones to withdraw cerebrospinal fluid (CSF) from around the spinal cord. The collected CSF is then analysed to look for the presence of tumour cells or any other abnormalities.

What are the treatment options for ependymoma in Singapore?

Ependymoma treatment typically depends on various factors, including the tumour's location, grade, and the patient's overall health. Here are the primary treatment modalities available for ependymoma patients in Singapore:

  • Surgery: usually the first line of treatment for ependymomas, with the primary aim of safely removing as much of the tumour as possible. While complete removal may not always be feasible due to tumour location, the aim is to reduce its size to alleviate symptoms and enhance prognosis.
  • Radiotherapy: radiotherapy, also known as radiation therapy, plays a critical role in treating ependymoma, particularly in cases where complete tumour removal is challenging or for high-grade ependymomas. Advanced radiation techniques, such as intensity-modulated radiation therapy (IMRT) or proton therapy, may be used to precisely target the tumour while minimising radiation exposure to healthy tissues.
  • Chemotherapy: chemotherapy may be considered for certain cases of ependymoma, especially for high-grade or recurrent tumours. Chemotherapy drugs are administered orally or intravenously, and they slow down or kill cancer cells.
  • Clinical trials: participation in clinical trials can be an option for some ependymoma patients. Clinical trials offer access to innovative treatments and therapies that are still being investigated for their effectiveness.

Frequently asked questions  

Are there long-term effects or complications associated with ependymoma treatment?

The long-term effects of ependymoma treatment vary based on factors such as the tumour's location, grade, and the specific treatments used. Potential complications may include neurological deficits, cognitive changes, and secondary cancers.

What are the survival rates for ependymoma?

Survival rates for ependymoma vary depending on factors such as the tumour's grade, location, and the extent of surgical resection. Generally, low-grade ependymomas have a more favourable prognosis than high-grade ependymomas.

Can ependymoma recur after treatment, and what are the signs of recurrence?

Ependymoma can sometimes recur after treatment. Signs of recurrence may include the return of previous symptoms, new neurological deficits, or abnormalities detected on follow-up imaging studies. Regular medical monitoring is essential to detect and address any potential recurrences promptly.

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