Medvet club X Neuroscience and Neurotechnology club
We always hope for the best for children and think nothing bad could happen to them. At worst we expect a few injuries while playing, or a minor fever, but little do we expect that children could be diagnosed with terminal diseases like cancer. Globally, approximately 400,000 children are diagnosed with cancer each year and brain tumours are the leading cancer-related cause of death in children - with DIPG being the most deadly and devastating as there is no effective treatment and barely any chance of survival. Only 30% survive a year after diagnosis, while there is only a <1% chance of survival after 5 years.
Diffuse Intrinsic Pontine Glioma is the most common paediatric brainstem tumour and is a highly aggressive paediatric brain tumour that grows in the Pons of the brain stem, which is found at the base of the brain, and controls the body’s most vital functions. They are glial tumours, meaning they stem from the brain’s glial tissue - tissue made up of cells that help support and protect the brain's neurons. These tumours are difficult to treat because they grow within the normal brain tissue which makes surgery to remove the tumour nearly impossible.
DIPG is graded by the nature of the tumour. Low-grade DIPGs are Grade 1 or Grade 2, while high-grade tumours, which are the most aggressive are Grade 3 (anaplastic) or 4 (glioblastoma multiforme). DIPG occurs when cell reproduction goes wrong. Researchers do not know what exactly causes a child to develop a DIPG tumour as there are no links between DIPG and any
specific inherited genetic variations or environmental factors but new research suggests that the formation of DIPG tumours may be related to brain development - the tumours tend to be linked to periods where development is rapid.
A Diffuse Intrinsic pontine Glioma affects the cranial nerves - 10 out of 12 of which originate in the brain stem, and these control sight, smell, taste, etc. DIPGs can thus cause significant neurological issues in the face. As the tumour grows quickly, symptoms often appear suddenly and worsen faster. The most common symptoms are issues with eye movement and swallowing. If the tumour causes hydrocephalus, which is an increase in pressure inside the skull due to the build-up of fluid, symptoms can then include headache, nausea, and vomiting.
For most brain tumours, the tumour is usually removed through surgery but DIPGs can’t be removed in this way as the pons is located in the centre of the brain, and to remove this tumour through surgery would mean that the surgeon would end up damaging the surrounding portions of the brain and total removal would be impossible as a DIPG has no defined margins - this invasive treatment would ultimately be extremely detrimental to a child’s growth and mental health.
Non-invasive treatments such as chemotherapy and radiation therapy are part of the standard treatment of a DIPG but are only used to stop the progression of the tumour. Radiation causes the tumour to shrink for roughly 70% of patients but the benefits of it are only temporary.
Conventional radiotherapy is the backbone of DIPG treatment and while radiation dramatically reduces symptoms, it does not increase the patient’s chance of survival and symptoms typically return after 6 to 9 months and continue to progress. Previously, hyperfractionated radiotherapy was used but did not increase survival rates among patients. Radiosurgery may be used as a part of treatment in selected cases. Chemotherapy and biological therapy combined with radiation therapy are being investigated as a treatment for DIPG. There have also been numerous clinical trials such as, “Intra-arterial Chemotherapy for the treatment of DIPG”, which began at Johns Hopkins.
As there is no known way to prevent DIPG in children and the fact that the use of radiation and chemotherapy can cause severe side effects in children of this age as well, there have been conversations about whether this pseudo-palliative care is ethical for young children and whether it allows them to maintain a good quality of life.
Bibliography:
1. “What Causes DIPG?” Michael Mosier Defeat DIPG Foundation, www.defeatdipg.org/dipg-
facts/overview/what-causes-dipg/. Accessed 9 Aug. 2022.
2. “Diffuse Intrinsic Pontine Glioma (DIPG).” Www.stjude.org, www.stjude.org/disease/diffuse-
intrinsic-pontine-glioma.html. Accessed 12 Aug. 2022.
3. “DIPG Statistics.” DIPG.org, dipg.org/dipg-stats. Accessed 8 Aug. 2022.
4. “DIPG Cancer: All about This Brain Tumor and Its Treatment.” Healthline, 2 May 2018,
www.healthline.com/health/cancer/dipg#stages. Accessed 9 Aug. 2022.
5. “Symptoms of DIPG.” Weill Cornell Brain and Spine Center, 10 Dec. 2012,
weillcornellbrainandspine.org/condition/diffuse-intrinsic-pontine-glioma-dipg/symptoms-dipg.
Accessed 12 Aug. 2022.
6. “What Is the Treatment for DIPG?” Michael Mosier Defeat DIPG Foundation,
www.defeatdipg.org/dipg-facts/overview/what-is-the-treatment-for-dipg/. Accessed 13 Aug. 2022.
7. “Childhood Diffuse Intrinsic Pontine Glioma - Dana-Farber Cancer Institute | Boston,
MA.” Www.dana-Farber.org, www.dana-farber.org/diffuse-intrinsic-pontine-glioma-dipg/.
Accessed 11 Aug. 2022.
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