These tumours are divided into four major types:
- Grade I: Subependymomas: Typically, slow-growing tumours.
- Grade II: Myxopapillary ependymomas: Typically, slow-growing tumours.
- Grade III: Ependymomas: The most common of the ependymal tumours. This type can be further divided into the following subtypes, including cellular ependymomas, papillary ependymomas, clear cell ependymomas, and tancytic ependymomas.
- Grade III: Anaplastic ependymomas: Typically, faster-growing tumours.
These tumours have these common characteristics:
- Ependymomas arise from the ependymal cells that line the ventricles of the brain and the center of the spinal cord. They are soft, grayish, or red tumours which may contain cysts or mineral calcifications.
- Can be a slow- or fast-growing tumour.
- In children, ependymomas most commonly arise in the back of the head (the posterior fossa), within the cerebellum.
- Posterior fossa tumours in children may be associated with spread to the spinal cord in 10-15% of cases
- The tumour may block the flow of the CSF (cerebral spinal fluid, which bathes the brain and spinal cord), causing increased intracranial pressure (called hydrocephalus).
- These tumours may occur in the brain or in the spinal cord.
- Ependymomas arise from the cells lining the ventricles (hollow channels) of the brain (most commonly the fourth ventricle).
- As these tumours grow and fill the ventricle, they obstruct the flow of cerebrospinal fluid (CSF) through the brain.
Common symptoms include, but are not necessarily limited to:
- Difficulty walking
- Fatigue and sleepiness
- Morning nausea and vomiting
- Neck pain or stiffness
- Problems with coordination
- Visual changes
- Symptoms of an ependymoma are related to the location and size of the tumour.
- In babies, increased head size may be one of the first symptoms.
- In older children and adults, headache, nausea and vomiting are the most common symptoms.
Treatment/Standard of Care
- Surgery is very important. The neurosurgeon will try to remove as much of the tumour as possible. A shunt may be needed to treat hydrocephalus caused by blockage of the ventricles.
- Moderately high-dose radiation therapy is commonly given to the posterior fossa alone, after surgery, if there is no sign of spread to other parts of the brain or spine.
- Chemotherapy has a role to play and may be used if the tumour cannot be removed by surgery completely at first. The tumour can shrink with chemotherapy and then surgery might be possible later.