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Diffuse Midline Glioma (DMG)

Diffuse Midline Glioma (DMG) Brain Tumor

Diffuse midline glioma (DMG) is a rare type of cancerous brain tumor. It's most common in children between the ages of 5 and 10 years old, though it can also affect adults. The name means:

 - Diffuse: The cancer cells have spread beyond the tumor and into healthy brain tissue.
 - Midline: The tumor is found in the middle section of the central nervous system, usually the            thalamus, spinal cord, or brainstem, also called the pons.
 - Glioma: The tumor grew from glial cells, which are "helper" cells in the white matter of the                brain. This may also include certain categories of Diffuse Intrinsic Pontine Glioma (DIPG).
 - Source: MayoClinic.org
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DIPG Changed to Diffuse Midline Glioma (DMG)

DIPG was reclassified as DMG when it was found that DIPG-like tumors, defined by similar histology and/or the H3 K27M mutation, could be found in other parts of the brain than the pons. For a long time, since 2016, there was only DIPG because prior classifications mostly relied on location in the pons, age of diagnosis, and findings under the microscope.

However, as molecular testing evolved and pathologists realized that many DIPGs had H3 K27M mutations, they started testing other tumors that looked similar under the microscope for the H3 K27M mutation. And in adults, the pontine location is not as common; more common in the thalamus, midbrain, and spinal cord.

This really illustrates the importance of genetic testing, because now adults who have this mutation, despite not being in the classic pons location, can qualify for FDA-approved targeted medications, such as Dordaviprone, and for clinical trials (some GMG trials have eligibility up to 60 years old).

Best Practices for Patients/Caregivers/Families:

  • INSIST on a Biopsy, comprehensive NGS/Biomarker & Fusion testing, and a larger gene panel (400–600 Genes) for targeted brain cancer therapy at the BEGINNING of your treatment.
  • Cast a wide net and source different opinions/introductions to specialists from your own network or Google search to see where they converge as it relates to recommendations.  This helps you begin to build your initial treatment team.
  • Start Building Your List of Questions for your Healthcare Team.
  • DO ask for a second or third opinion. Know that access to healthcare does not mean “EXPERTISE.”
  • Start Building Your Treatment Options List. You want diagnostic and treatment options in your “Treatment Tool Kit.” Don’t let the Healthcare Provider brush you off when you bring an option to them for consideration.
  • Build a binder/have a system for cataloguing information related to diagnostic/treatment options, specialists, and clinical trials.
  • Contact a patient advocacy organization like the End Brain Cancer Initiative to help you navigate treatment options and clinical trials, schedule second opinions, introduce you to specialists, and help you access advanced diagnostics, treatment options, and clinical trials.
  • Seek a brain tumor center and/or a doctor (neuro-surgeon and neuro-oncologist) who routinely performs DMG brain tumor resections and biopsies, including in the brain stem, and treats DMG brain tumors, especially if you live in a rural setting.
    • INSIST on a referral to a specialist and seamless transition from your doctor.
  • If tissue is obtained during surgery or biopsy, ask how it will be preserved and whether enough tissue will be available for comprehensive molecular testing and potential future research. Tissue must be saved via frozen process vs. paraffin for comprehensive NGS Testing to be done.
  • Continue to ask about clinical trials and what other options there are if eligibility change

Need Help Understanding Your Options?

EBCI Patient & Resource Navigators can help patients and caregivers better understand DPG results, explore testing resources, identify potential clinical trial opportunities, and prepare questions to discuss with their healthcare team.

Shreya Prakash
4235-444-2215
shreya@endbraincancer.org

This information is intended for educational purposes and is not a substitute for medical advice. Patients should discuss testing results and treatment decisions with their qualified healthcare provider.
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ENDBRAINCANCER INITIATIVE | CHRIS ELLIOTT FUND

Enhancing patient outcomes by expanding FDA-approved treatment modalities and fueling research in the pharma/bio/life sciences, device & diagnostic industries and by closing the existing GAP from initial diagnosis to IMMEDIATE AND EXPANDED ACCESS to specialists, researchers, advanced & innovative treatments, clinical trials and critical care with the ultimate goal of improving patient outcomes through updating and improving WHO & NCCN Guidelines and clinical practices related to Standard of Care for brain cancer patients.

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