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Feb 225 min read

Brain Cancer Breakthroughs: Advancements in Diffuse Intrinsic Pontine Glioma Research



Brain cancer affects thousands of people annually, including children. Brain and spinal cord tumors are the second most frequent type of cancers in children, after leukemia, accounting for about 25% of childhood cancer cases. One of the most dangerous forms is Diffuse Intrinsic Pontine Glioma (DIPG).

Diffuse Intrinsic Pontine Glioma (DIPG) is a highly aggressive brain tumor that starts in the brainstem, the lowest part of the brain just above the neck and connected to the spinal cord, more specifically in the pons. This region is responsible for breathing, heart rate along with the nerves and muscles that help with our sight, hearing, walking, talking, and eating.

The American Childhood Cancer Organization (ACCO, 2018) says that on average, the five-year survival rate (meaning the number of children who survive more than five years after the end of treatment) across all types of brain and spinal cord tumors (including both malignant and benign) is very good: 3 out of 4 children diagnosed with a brain tumor will survive longer than five years.

Unfortunately, the situation is not the same for patients suffering from DIPG. After diagnosis, 90% of kids die within 2 years, making the overall survival rate less than 1% (ChadTough, n.d.). 

According to the Mithil Prasad Foundation (2020), the first DIPG case reported was in the 1920s. The patient was diagnosed after showing classic DIPG symptoms and sadly died within a few months. That is a natural occurrence if the disease is not treated at all. Symptoms and clinical signs include odd eye movements, slurred speech, difficulty swallowing, trouble maintaining balance, drooping of one part of the child’s face, headaches (which are often worse in the morning) accompanied by nausea and vomiting (ACCO, 2012, as cited in Raabe & Cohen, n.d.). Since the beginning of childhood cancer research in the 1970s (Baxter & Blaney, n.d.), researchers and organizations have tried to find the cause: 

Researchers do not yet know what causes DIPG. Like most cancers, DIPG occurs when something goes wrong with the process of cell reproduction. However, unlike many other cancers, there is no evidence that indicates DIPG is caused by environmental factors (exposure to chemicals or radiation), or specific inherited genetic variations (The DIPG / DMG Resource Network, n.d.).

At the same time, Cleveland Clinic (n.d.) says a child may have a higher risk of getting DIPG if they have a rare disorder called Li-Fraumeni Syndrome. They also mention that cell changes or mutations (like the protein  H3K27M) can cause glial cells to become cancer cells (“What causes diffuse intrinsic pontine glioma (DIPG)?,”).

In terms of treatment, radiation therapy (five daily sessions per week for a total of 30 to 33 sessions) is the best option since the high-energy X-rays reduce the size of the tumor and the pressure on the brain (Souweidane, 2022). On average, this treatment keeps the cancer away for six months, and when the tumor comes back, the patient goes through repeat radiation therapy (five daily sessions per week - with a different dose this time - for 10 sessions). It is known that for most brain tumors, surgery is the first option. However, in DIPG, the tumor grows so quickly in the brainstem (which controls breathing) that it is impossible for it to be surgically removed. The same goes for chemotherapy; anti-cancer drugs cannot cross the blood-brain barrier and kill the cancerous cells.

The good news is that there have been some successful clinical trials for DIPG in the past few years. Brain Research UK (2021) has part-funded childhood brain cancer research in which scientists have discovered a new combination of drugs, using artificial intelligence-enhanced tools that could lead to a cure for DIPG. In 2014, Professor Chris Jones and his team discovered a mutation called ACVR1 which is not seen in any other types of cancer. Interestingly, the same mutation is found in the Stone Man Syndrome (a genetic mutation that turns muscles into bones) which means the drugs used for this syndrome could be used to treat DIPG too. Vandetanib is a drug that acts against the ACVR1 gene, but it has difficulty breaking the blood-brain barrier. Fortunately, the Brain Research UK Organization also said: 

The research team at the Institute of Cancer Research, London, led by Professor Chris Jones, found that a drug called Everolimus could enhance vandetanib's capacity to pass through the blood-brain barrier in order to treat the cancer.

After combining the two drugs and testing them on the brains of mice with DIPG, they found out that the amount of Vandetanib was increased by 56% and the survival rate for mice was boosted by 14% compared to those receiving a control treatment. Moreover, the two drugs mentioned are already approved by the European Medical Association and the US Food and Drug Administration. Researchers and Professor Jones are ready to test this drug combination in clinical trials with young patients in the near future.

These discoveries are important from many different perspectives. First, they proved that the use of artificial intelligence can help in the most ‘hopeless’ cases and that people can make incredible things if they use it for the greater good. Second, they show how important DIPG research funding is; it led to the development of new treatment options which can potentially improve the patients’ survival rate. Lastly, they could contribute to raising more public awareness, greater advocacy efforts, and eventually better outcomes for the children and their families. 


 
Alexandra Badescu is a high school senior from Bucharest, Romania. She is an aspiring neuroscientist who loves origami, jazz, and reading biographies. By the end of this year, she wants to apply to university in the United States and start a new life across the ocean.

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