It is a tumour located in the pons (middle) of the brain stem. The brain stem is the bottom-most portion of the brain, connecting the cerebrum with the spinal cord. The majority of brain stem tumours occur in the pons (middle brain stem), are diffusely infiltrating (they grow amidst the nerves), and therefore are not able to be surgically removed.
Glioma is a general name for any tumor that arises from the supportive tissue called glia, which help keep the neurons ("thinking cells") in place and functioning well. A brainstem glioma is a cancerous glioma tumor in the brainstem. The brain stem contains all of the "wires" converging from the brain to the spinal cord as well as important structures involved in eye movements, face and throat muscle control and sensation.
We estimate that approximately 300 children are diagnosed each year in the US with DIPG (interpolating this to the world population will lead to a few thousand globally).
Over 90% die within 12 months, 97% within three years.
Usually diagnosed in children from ages 5 to 10.
Diffuse Intrinsic Pontine Gliomas account for about 10 out of every 100 brain tumours in children but constitutes about 80% of deaths resulting from brain tumours.
We don’t know what causes a diffuse pontine glioma. There is no way to predict that a child will get brain cancer.
Some DIPG symptoms are muscle weakness on one side of the body, swallowing problems, speech problems, crossed eyes, drowsiness, hearing loss, and personality changes. The brainstem has cranial nerves that control many of these functions. The tumours affect these nerves and cause symptoms.
Diffuse Intrinsic Pontine Gliomas are difficult to treat because the tumour cells grow in between and around normal cells. It is impossible to remove a tumour in this area because it interferes with the functioning of this critical area of the brain.
30 sessions done under general anaesthesia is the only proven existing treatment for DIPG and produces a temporary improvement in symptoms (average of 3 months) which eventually reappear and develop at a much faster, more aggressive pace.
Children with DIPG must endure one experimental trial after another, usually suffering terrible side-effects. Ultimately, it is with enormous cruelty that this disease takes its final toll. DIPG spares children of their cognitive abilities, allowing them to remain fully aware of their decline while robbing them of their motor functions resulting in partial paralysis, loss of voice, sight and finally their ability to eat and breathe.