What is DIPG?
What Kind of Cancer is DIPG?
DIPG is a type of childhood brain cancer that is most common in children with fewer than 10% of children diagnosed with this aggressive type of brain tumor surviving beyond two years from the date of their diagnosis. DIPG brain tumors are developed from glial cells, or neurological cells, common in the brain and central nervous system. Glial cells were once thought of as “non-functional glue for neurons” however, it has been discovered through several decades of research, that they are more functional and important than originally thought. Although, there are still many questions about how these cells function under various conditions.
What Does DIPG Stand For?
DIPG stands for Diffuse Intrinsic Pontine Glioma and is also called Pontine Glioma or Brainstem Glioma. It may sound like a long, complicated name, but it actually describes aspects of the tumor such as how it grows (diffuse), where it grows (pontine), and the type of cells it originates from (glioma). The word “intrinsic” means “a point of origin” or, essentially, “in.”
What is the Survival Rate of DIPG?
The survival rate of DIPG is very low, with the disease currently being considered a fatal cancer.
The chances of surviving 2 years following a diagnosis are about 10% while the chances of surviving beyond 5 years is about 2%, which happens to be up by 1% since 2018.
Nearly half of patients diagnosed with this cancer died within 9 months of being diagnosed.
How Common is DIPG?
DIPG is considered an aggressive and quick-growing cancer. DIPG tumors make up about 10-20% of all childhood brain tumors. According to DIPG.org, brain tumors are the most common form of cancer in children under the age of 15 and diffuse intrinsic pontine gliomas make up nearly 80% of all pediatric brainstem cancers.
Is DIPG on the Rise?
In the United States, there are between 300-400 cases of DIPG annually which calculates out to 1-2 cases per population of 100,000.
Some specific locations within the U.S. have seen a rise, but the data isn’t significant enough to determine whether the rise in incidents was merely a coincidence or linked to a common cause.
What Causes DIPG?
There are many different kinds of cancers and, as a result, numerous causes.
Some cancers are inherited through genes passed from their parents while others are caused by environmental factors.
At this time, researchers cannot confirm a direct cause of DIPG. However, this type of cancer occurs, like many others, when issues with cellular reproduction arise and recent studies suggest that it may correlate to events that occur during the brain’s development. Hence, the prominence of this cancer among children, when their brains are developing.
Is DIPG Inoperable?
Surgery is very rarely used to treat DIPG due to the location of the tumor in the brain.
It is difficult to remove the tumor, given the extremely high risks associated with the area of the brain where the tumors are commonly located- in or near the brainstem.
Biopsies are even challenging to complete for the same reason, but are possible, depending on the tumor’s growth.*
What are the Treatment Options for DIPG?
Because surgery is rarely a treatment option, a combination of radiation therapy and steroids are typically used to treat DIPG.
These two treatments are the first approach used, as DIPG has not responded well to chemotherapy drugs in the past and has done little to impact survival rates.
Treatment for DIPG has gone unchanged for nearly 40 years. With no known treatment, families are left to clinical trial drugs that are unproven.
When was DIPG Discovered?
There does not appear to be a definitive date for the first diagnosis of DIPG, but there is evidence of probable cases dating back nearly a century.
In addition, according to Wikipedia, Karen Armstrong, the daughter of Neil Armstrong and Janet Elizabeth Shearon passed away from DIPG at the age of 3 years old in 1962.
What are the Signs and Symptoms of DIPG?
- Difficulty balancing
- Facial drooping
- Slurring of speech
- Trouble swallowing
- Erratic eye movements
How is DIPG Diagnosed?
If DIPG is suspected, a physician will order imaging that includes an MRI (magnetic resonance imaging) and a CT (computed tomography) scan to check for the presence of tumors. If tumors are identified, biopsies may be gathered for further examination – if safe – and the tumor(s) graded from 1-4, based on their type and level of aggression.
Is DIPG Hereditary?
There are no signs that point to DIPG being passed through inherited traits and the underlying cause of DIPG is still unknown.
What is the Team Jack Foundation doing to help?
The mission of the Team Jack Foundation is to fund impactful childhood brain cancer research and work to create national awareness for the disease. Since 2013, we have committed $8.375 million to nine different research projects. Of those nine projects, two are dedicated to specifically studying DIPG. To learn more about these projects visit our research page. Funding for research is lacking, in fact, less than 4% of the federal government’s research investments go toward childhood cancer and even less for childhood brain cancer. Private foundations like Team Jack are left to carry the torch. If you would like to help us in our efforts, please consider making a donation.
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Day One Announces Positive Initial Data from Pivotal FIREFLY-1 Trial of Tovorafenib (DAY101) in Relapsed Pediatric Low-Grade Glioma (pLGG)
Data show an overall response rate (ORR) of 64% and clinical benefit rate (CBR) of 91% in the first 22 evaluable patients treated with monotherapytovorafenib Topline results from the full FIREFLY-1 trial population expected in Q1 2023 Day One plans to initiate a...
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The 9th Annual Team Jack Gala was held on February 26 in Lincoln, Nebraska raising $635,000 for research. Catch up on the event with the digital event program.