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Ependymoma

40% of all cancers spread to the brain

What is an ependymoma?

An ependymoma is a rare type of brain tumour that develops from the ependymal cells that line the passageways containing cerebral spinal fluid. They are therefore found in the walls of the ventricles or the spinal cord central canal.

Ependymoma can be found in any part of the brain or spine but in children, they are more common in the cerebellum, in an area called the posterior fossa, a small space located at the back of the brain near the brain stem.

Ependymomas represent approximately 1.9% of all primary brain and central nervous system tumours and around 25% of spinal tumours. Ependymomas can also form in the choroid plexus.

Ependymoma brain tumours tend to be diagnosed in children around the age of five years old, and adults of around 45 years. Spinal ependymomas are less common in children and most commonly diagnosed in adults aged around 45 years.

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Are ependymomas classified as low-grade (benign) or high-grade (malignant) brain tumours?

An ependymoma can be classified as a grade 1 or 2 low-grade (slow-growing) tumour or a grade 3 high-grade (malignant) tumour, usually referred to as anaplastic ependymoma.

Low-grade ependymoma tumours (grade 1 and grade 2) are more common in the spine. High-grade or anaplastic ependymomas (grade 3) occur most often in the brain.

Ependymomas are partly classified based on their location in the brain: either supratentorial region or posterior fossa.

They are further divided into distinct molecular subgroups that reflect differences in age of onset, gender predominance, and their expected response to treatment. For example, the most common and aggressive subgroup, posterior fossa ependymoma group A, occurs primarily in young children. Posterior fossa ependymoma group B tumours display more favourable clinical outcomes.

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Other types of ependymoma

Anaplastic ependymoma

This is classified as a grade 3 ependymoma. ‘Anaplastic’ is a term used to describe a number of different types of tumour, meaning that at this stage the tumour is formed of cancer cells that are dividing rapidly and have little or no resemblance to normal cells.

Cellular ependymoma

A cellular ependymoma is a form of ependymoma that has a particular appearance under a microscope, often with less obvious pseudo-rosette or rosette formation seen amongst the cells when compared to other forms of ependymomas. It is classified as a grade 2 ependymoma.

Clear cell ependymoma

A clear cell ependymoma is a rare form of ependymoma, usually classified as a grade 2 tumour but can also be grade 3. It is named after the way that the cells look under a microscope (a histological classification).

Mxyopapillary ependymoma

A mxyopapillary ependymoma tends to occur in the lower part of the spinal column and is classified as a benign, low-grade or grade I.  

Papillary ependymoma

A papillary ependymoma is a rare form of ependymoma, usually classified as a grade 2 tumour but can also be a grade 3.

Subependymoma

A Subependymoma is a type of ependymoma that has a distinctive appearance under a microscope (histologic variant), and can be found within the supratentorial region (ST) or posterior fossa (PF) regions of the brain. These account for the majority of tumours in the molecular subgroups ST-EPN-SE and PF-EPN-SE, respectively. They are considered to be a benign, low-grade or grade I brain tumour.

Tanycytic ependymoma

A tanycytic ependymoma is a form of grade 2 or grade 3 ependymoma that can be seen to have elongated tanycte cells when viewed under a microscope. Tancytes are common progenitor cells of both ependymal cells and astrocytes: in other words, they could potentially become either an ependymal or an astrocyte cell. 

 

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What causes an ependymoma?

Ependymomas develop from ependymal cells, which are a type of glial cell. Glial cells provide the structure around neural cells within the brain, and help to keep the neural cells healthy. When the DNA within an ependymal cell becomes damaged, the cell can replicate itself in an uncontrolled way, without apoptosis. This uncontrolled growth is what leads to the formation of a tumour.

Ependymoma tumours can occur as a result of a genetically inherited condition called neurofibromatosis 2. Find out more about this condition here.

What are the symptoms of an ependymoma?

The symtoms of an ependymoma will depend upon where it has arisen in the brain. Find out more here.

What is the best treatment for ependymoma?

Children and adults with ependymoma respond differently to treatment and therefore tend to be treated in different ways.

The first line treatment for both adult and paediatric ependymoma is surgery. For some patients, particularly those for whom the surgery successfully removes the tumour, no further treatment is offered, although close monitoring using regular MRI scans is highly recommended.

For paediatric patients with ependymoma, the surgery is sometimes done in a staged way so that some of the tumour is removed, and then the tumour is shrunk further using radiotherapy, chemotherapy or a combination of both treatments. Another operation may then be undertaken to remove more of the tumour.

For some adult patients, radiotherapy is offered once they have recovered from the surgery.

It is unusual for adults to be offered chemotherapy unless they have a grade 3 ependymoma. It is more common for children to be offered chemotherapy for their ependymoma brain tumour.

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How will we find a cure for ependymoma?

Research we are funding across all of our Centres of Excellence will help lead towards finding a cure for a wide range of brain tumours.

Our University of Plymouth Low-Grade Brain Tumour Research Centre of Excellence is Europe’s leading research institution for low-grade brain tumours. The teams there have a strong focus on both Neurofibromatosis 2 and brain tumours that have an NF2 gene mutation.

The team of research and clinical experts at our Brain Tumour Research Centre of Excellence at Imperial College, London, work closely with the University of Plymouth Centre of Excellence on certain aspects of research into low-grade brain tumours, including those with an NF2 gene mutation.

Scientists at our Brain Tumour Research Centre of Excellence in the University of Portsmouth also collaborate with the University of Plymouth Centre of Excellence on some aspects of low-grade brain tumour research. They have developed models of the blood-brain barrier that support research into drug therapies for all types of brain tumours.

Pioneering research at our Brain Tumour Research Centre of Excellence at Queen Mary University of London has developed methods of studying stem cells that could potentially transfer across all types of brain tumour.

We also fund BRAIN UK at Southampton University, the country’s only national tissue bank providing crucial access to brain tumour samples for researchers from the archives of clinical neuroscience centres in the UK, effectively covering about 90% of the UK population, which is an essential component in the fight to find a cure for brain tumours.

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