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WHAT IS DIPG?

What is DIPG?

DIPG is a type of cancer that affects children, most commonly between the ages of five and 10 years. It’s considered a type of diffuse brain tumor affecting the pons region of the brain, which is part of the brainstem. The brainstem is located at the base of the brain and regulates our body’s involuntary activities such as breathing and our heartbeat. Without the brain stem, our body would lose its ability to essentially run on autopilot.

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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.”

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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.

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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.

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Is DIPG on the Rise?

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.

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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.

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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.*

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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.

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What are the Signs and Symptoms of DIPG?

  • Difficulty balancing

  • Facial drooping

  • Slurring of speech

  • Trouble swallowing

  • Erratic eye movements

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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.

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