A Comprehensive Guide to Understanding Diffuse Intrinsic Pontine Glioma (DIPG) and Diffuse Midline Glioma (DMG)

Diffuse Intrinsic Pontine Glioma (DIPG) is a rare and aggressive brainstem glioma which usually affects children. This pediatric brain tumor is located in the brain stem and is notoriously difficult to treat. This comprehensive guide will provide an in-depth look into DIPG, its symptoms, diagnosis, and the latest treatment options.

Diffuse Midline Glioma (DMG) is a newer pathology term used for gliomas that invade normal tissue in the “midline” of the brain. The midline includes multiple structures (the thalamus, the brainstem, and the spinal cord). Most biopsied DIPG tumors are diagnosed as DMG. Additionally, you can have a DMG outside of the brainstem that is not a DIPG.

Table of Contents

  1. What is Diffuse Intrinsic Pontine Glioma (DIPG)?
  2. Symptoms of DIPG
  3. Diagnosing DIPG
  4. Treatment Options for DIPG
  5. Coping with a DIPG Diagnosis
  6. Resources and Support
  7. Hope from Research

1. What is Diffuse Intrinsic Pontine Glioma (DIPG)?

Diffuse Intrinsic Pontine Glioma (DIPG) is a type of brainstem glioma, meaning it is a tumor that arises from the supportive cell (glial) tissue in the brainstem. The brainstem is responsible for essential neurological functions such as respiration, heart rate, and motor coordination. DIPG accounts for approximately 10-15% of all childhood brain tumors, with a majority of cases occurring in children aged 5-9 years old.

The aggressiveness of DIPG tumors is determined by their infiltrative nature and location within the brainstem, which makes complete surgical removal nearly impossible. Prognosis for children with DIPG remains poor, with survival rates remaining relatively unchanged over the past few decades. However, ongoing research and clinical trials are providing improved treatment options and are
making progress in ultimately changing the outlook for DIPG patients.

Key Facts & Figures:

  • DIPG accounts for 10-15% of childhood brain tumors.
  • Most commonly affects children aged 5-9 years old.
  • Infiltrative nature and brainstem location make treatment difficult.

By the Numbers

#1 cause of death by disease for children is cancer; the most lethal form is brain cancer.

Facts and Prognosis

2. Symptoms of DIPG

The symptoms of DIPG vary depending on the specific area of the brainstem affected by the tumor. As symptoms progress, DIPG may also lead to more significant neurological complications, such as difficulty breathing and reduced consciousness.

Common symptoms include:

  • Difficulty with eye movements, double vision
  • Facial weakness or asymmetry
  • Difficulty with balance and coordination
  • Problems with chewing and swallowing
  • Weakness in the arms or legs
  • Nausea and vomiting
  • Headaches, particularly in the morning

Why and How

An overview of how the disease presents symptomatically, and some theories about why.

Effects and Causes

3. Diagnosing DIPG

Diagnosis of DIPG typically involves a combination of physical examination, neuroimaging, and biopsy when appropriate. Advanced imaging techniques such as magnetic resonance imaging (MRI) can provide detailed information about the tumor’s location, size, and infiltration into surrounding tissues. While a biopsy may be difficult to perform, it is possible in many cases with an experienced neurosurgeon.

Staging for DIPG tumors is based on the extent of infiltration and symptoms present. Accurate diagnosis and staging are crucial for determining the most appropriate treatment plan.

4. Treatment Options for DIPG

The primary treatment options available for children with DIPG include radiation therapy, chemotherapy, and participation in clinical trials. Due to the tumor’s infiltrative nature, surgery is typically not an option for DIPG treatment.

4.1 Radiation Therapy

Radiation therapy is considered the only approved standard of care for children with newly diagnosed DIPG. It is the most effective initial treatment in relieving symptoms and potentially slowing tumor growth. Radiation therapy is administered by radiation oncologists and involves directing targeted beams of radiation at the tumor site, with the aim of damaging the cancer cells while minimizing harm to healthy tissue.

4.2 Chemotherapy

Chemotherapy drugs are designed to kill cancer cells, but their effectiveness in treating DIPG has historically been limited. However, ongoing research and clinical trials are exploring new chemotherapy drugs, drug combinations, and biologic agents that may be more effective in managing DIPG. A patient’s eligibility for these treatments may depend on individual factors, including age, general health, and tumor characteristics.

4.3 Clinical Trials

Children with DIPG might have the option to participate in clinical trials testing new treatment approaches. These trials are a critical part of the process of developing new therapies and can potentially offer children access to otherwise unavailable treatment options. It’s essential to discuss potential trial participation with your child’s medical team to understand the potential risks, benefits, and eligibility criteria.

5. Coping with a DIPG Diagnosis

A diagnosis of DIPG can be devastating for both the affected child and their family. It is crucial to find adequate support, including:

  • Regular communication with your medical team
  • Support groups for families affected by childhood cancer
  • Engaging with pediatric palliative care teams for medical, emotional, and practical support
  • Seeking professional psychological or counseling assistance if necessary

What to Expect

Start with this definitive guide to best practices and treatment options for DIPG/DMG.

Best Practices

6. Resources and Support

Understanding the complexities of DIPG is crucial for parents and caregivers considering treatment options and seeking support. By staying informed, you can better advocate for your child’s care and participate in choosing the most appropriate treatment options. Whether through standard of cae treatments, clinical trials, or palliative care, the ultimate goal is to provide comprehensive support and care for children living with this difficult diagnosis.

My DIPG Navigator is a nationwide program designed to provide free, individualized guidance for patients facing a DIPG (diffuse intrinsic pontine glioma) or DMG (diffuse midline glioma) diagnosis. Deeply experienced in pediatric oncology, our Registered Nurses share options and expectations and connect families to the resources essential to making the most informed decisions throughout their cancer journey. My DIPG Navigator is a program developed and run by the ChadTough Defeat DIPG Foundation with support from their Navigator Partners and Family Partners.

1-on-1 Support for Families

Designed by families who have faced DIPG/DMG, this is the unique FREE resource they wish had existed during their own fight.

My DIPG Navigator

7. Hope from Research

Every day, researchers get one step closer to a cure. ChadTough Defeat DIPG Foundation is the largest private funder of DIPG/DMG research in the world. In addition to supporting some key clinical trials like Car-T cell and the Diffuse Midline Glioma – Adaptive Combinatorial Therapy (DMG-ACT) trial, the foundation attracts new talent to the field through pre-doctoral and post-doctoral Fellowship grants, as well as New Investigator grants. It also supports hypothesis-driven research projects from seasoned DIPG/DMG researchers that represent an innovative approach to a major challenge in DIPG research through its Game Changer grants.

“A significant number of DIPG patients are now getting 6 months or more of additional survival from newer therapies, including ChadTough Defeat DIPG funded projects like ONC201 and CAR T.”

Dr. Carl Koschmann
Clinical Research Director
Chad Carr Pediatric Brain Tumor Center