Our understanding of what a Diffuse Pontine Glioma is…
Diffuse Intrinsic Pontine Glioma
Pontine gliomas are malignant (cancerous) tumours that originate from the part of the brain known as the brain stem (pons). They develop from cells called astrocytes. Astrocytomas are the most common type of glioma. You will often hear the terms astrocytoma and glioma used interchangeably. In children, 80% of brain stem tumours are pontine gliomas.
If a biopsy is carried out they are usually found to be high-grade gliomas and are classified according to the grade of aggressiveness (how quickly they grow) as either anaplastic astrocytomas (grade III) or glioblastoma multiforme (GBM). These tumours grow amongst the normal nerve cells of the brain stem making them impossible to remove surgically as the brain stem is a vital area of the brain that controls many body functions.
Download a factsheet on Brainstem Glioma (pdf, 678Kb)
Who gets pontine glioma?
Around 35 children a year develop pontine glioma in the UK. This is around 10-15% of all childhood brain tumours. They rarely occur in adults. Like most brain tumours, the cause of pontine gliomas is unknown. These tumours can develop at any time but the most common ages are between 5 and 10 years of age.
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Signs and symptoms
The symptoms are related to the internal pressure that the tumour applies on the brain stem. Occasionally they can cause increased pressure in the head (raised intracranial pressure). Rarely they spread into the spine.
Symptoms can include:
If there is increased pressure in the head:
If the tumour spreads to the spine:
How is it diagnosed?
When a child arrives at a hospital with clinical symptoms suggestive of a brainstem glioma, the MRI is the most appropriate scan to give a diagnosis. If the brainstem tumour is diffuse usually no other tests will be needed before treatment since the diagnosis is almost certain.
What is the treatment for 'Diffuse brainstem gliomas'?
Radiotherapy: This is the only effective treatment in these particular tumours. This treatment is given just to the area of the tumour over a 6 to 7 week period. New radiotherapy techniques such as hyperfractionated radiotherapy (delivering a higher dose in 2 treatments per day) have not shown a benefit over classical radiotherapy (one treatment per day).
Chemotherapy: For diffuse brainstem glioma, studies have shown no convincing evidence that chemotherapy will be helpful. However, there are ongoing trials on selected chemotherapies.
Steroids: Due to the pressure on the vital cells in the brainstem, children with brainstem tumours are often very ill at the time of presentation. It is common to prescribe steroids to help to relieve some of their symptoms, as they reduce any extra swelling around the tumour and so help to relieve pressure.
Regrettably, even after all of the original symptoms have disappeared, there is very little chance of cure for children with diffuse brainstem gliomas. In most cases, the symptoms come back or get worse after a symptom free time usually measured in months not years. Most children die within a year of diagnosis.