Ependymoma

Overview


Tumour Group: Pediatric Brain Tumours and Non-Malignant Brain Tumours
WHO Grade: Grade I (mysopapillary ependymoma – this is often a spinal cord tumour), Grade II (ependymoma) or as Grade III (anaplastic ependymoma).
Prevalence/Incidence: Ependymomas account for:
  • 2-9% of all neuroepithelial tumours
  • 6-12% of all intracranial tumours in children
  • Up to 30% of tumours in children younger than three years of age
Typical Age Range: Common in children, and among men and women in their 40s and 50s. Occurrence peaks at age 5 and again at age 34.

Contents
Description of Tumour
Symptoms
Treatment / Standard of Care
Prognosis
References

Description of Tumour


  • 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.

Symptoms


Common symptoms include:

  • Difficulty walking
  • Fatigue and sleepiness
  • Headaches
  • 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. Irritability, sleeplessness and vomiting may develop as the tumour grows. 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.

Prognosis  


A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. Since every person is different, please take time to talk with your health care team about how this information applies to you.

By clicking on 'Expand,' a statistic on the prognosis for ependymomas will be shown.

Expand for Prognosis Information

For brain tumour patients, a prognosis depends on several factors, which can include age and other health issues, the size of the tumour, its molecular profile, the type of tumour, how much can be removed and its response to treatments such as chemotherapy and radiation therapy.

For childhood cases alone, the 5-year overall survival rate for ependymoma is 60-70% if treated with attempted total tumour resection and post-operative high-dose radiation therapy.

References


 Adult Brain Tumour Handbook 6th Edition. Brain Tumour Foundation of Canada. 2012.

 "Ependymoma." Ependymoma. National Brain Tumour Society, n.d. Web. 11 July 2013.

"Ependymomas in Children." Ependymomas (Glioma) in Children. John Hopkins Medicine, n.d. Web. 11 July 2013.

Louis, David N. WHO Classification of Tumours of the Central Nervous System. Lyon: International Agency for Research on Cancer, 2007. Print

"National Cancer Institute." Childhood Ependymoma Treatment (PDQ®) -. N.p., n.d. Web. 21 Sept. 2013.

Non-Malignant Brain Tumour Handbook. Brain Tumour Foundation of Canada. 2011.

Pediatric Brain Tumour Handbook 6th Edition. Brain Tumour Foundation of Canada. 2012.

Image credited to http://radiopaedia.org/articles/ependymoma

 

You can also download this information as a Information Sheet on Ependymoma (pdf).

 

In spring 2005, Jennifer began experiencing strange sensations at the back of her head whenever she would get up too quickly and her headaches were getting worse. A CT scan that fall uncovered the reason behind all her symptoms - Jennifer had an ependymoma. 

Almost ten years later, Jennifer has regular MRIs to monitor the tumour and in her words, "things are good!" Watch Jennifer's story of hope in this short video >>

 

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