Ependymoma

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.

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.

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. 

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.