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What is DIPG?

DIPG, or Diffuse Intrinsic Pontine Glioma, is a highly aggressive and rare type of brain tumor that primarily affects children. It is classified as a high-grade glioma and originates in the pons region of the brainstem. The pons is responsible for coordinating vital functions such as breathing, blood pressure, and balance.

Characteristics of DIPG include:

  • Diffuse Growth: DIPG tumors infiltrate and spread throughout the brainstem, making them extremely challenging to treat surgically. Due to their diffuse nature, complete surgical removal is typically not possible.
  • Glioma: DIPG arises from abnormal glial cells, which are supportive cells in the brain.
  • Intrinsic: The tumor develops within the brain tissue itself and does not typically spread to other parts of the body.

DIPG most commonly affects children between the ages of 5 and 10, although it can occur in older children and occasionally in adults. The exact cause of DIPG is still unknown, and there are no known risk factors or preventive measures.

Symptoms of DIPG may vary depending on the location and size of the tumor but often include:

  • Difficulties with eye movements, facial control, or swallowing.
  • Weakness or paralysis in the limbs.
  • Problems with coordination, balance, or walking.
  • Headaches.
  • Changes in behavior, personality, or cognitive abilities.

Unfortunately, the prognosis for DIPG is generally poor, and the average survival rate is around 9 to 12 months from the time of diagnosis. Traditional treatments like surgery and radiation therapy have limited effectiveness due to the tumor’s location and aggressive nature. Chemotherapy has shown limited benefits in some cases, but there is a need for more effective treatment options.
Research efforts are ongoing to better understand the biology of DIPG and develop innovative therapies. Clinical trials exploring targeted therapies, immunotherapy, and other novel approaches are being conducted in an effort to improve outcomes for patients with DIPG.

Treatment for DIPG

The treatment options for Diffuse Intrinsic Pontine Glioma (DIPG) are limited due to the tumor's location in the brainstem and its aggressive nature. Currently, there is no standard curative treatment for DIPG, and the primary goal of treatment is to alleviate symptoms and improve the quality of life for affected children.
  1. Radiation Therapy: Radiation therapy is the mainstay of treatment for DIPG. It is typically administered to the entire tumor area and surrounding brainstem over a period of several weeks. Radiation helps reduce swelling and temporarily shrink the tumor, which can alleviate symptoms and improve neurological function. However, the tumor eventually becomes resistant to radiation, and the disease progresses.
  2. Clinical Trials: Clinical trials are an important avenue for exploring new treatment options for DIPG. These trials may involve novel therapies, targeted treatments, immunotherapies, or combinations of therapies. Participation in clinical trials allows patients to access experimental treatments that may offer potential benefits.
  3. Chemotherapy: The role of chemotherapy in treating DIPG is limited. Traditional chemotherapy drugs have shown limited effectiveness as standalone treatments. However, certain chemotherapy agents, such as temozolomide, may be used in combination with radiation therapy or as part of experimental treatment protocols.
  4. Experimental and Targeted Therapies: Researchers are actively investigating new therapies and approaches for DIPG. This includes targeted therapies aimed at specific genetic mutations or alterations found in some DIPG tumors. Examples include targeting specific signaling pathways or epigenetic changes within the tumor cells. These therapies are still in the experimental stages and typically accessed through clinical trials.
  5. Supportive Care: Alongside treatment, supportive care plays a vital role in managing symptoms and improving the quality of life for children with DIPG. This includes palliative care, pain management, physical therapy, occupational therapy, and psychological support for both the patient and their family.

It is essential for families of children with DIPG to work closely with a multidisciplinary team of healthcare professionals, including pediatric neuro-oncologists, radiation oncologists, and supportive care specialists, to develop an individualized treatment plan based on the specific needs and circumstances of the child. Participation in clinical trials can also provide access to emerging treatment options and contribute to advancements in DIPG research.

Here are some resources that provide information, support, and resources for Diffuse Intrinsic Pontine Glioma (DIPG):

  1. DIPG Collaborative (dipg.org): The DIPG Collaborative is a global consortium of medical professionals, researchers, and foundations dedicated to finding a cure for DIPG. Their website offers information about DIPG, ongoing research, clinical trials, and resources for families.
  2. The Cure Starts Now Foundation (thecurestartsnow.org): This foundation focuses on pediatric brain cancer research, including DIPG. Their website provides information about DIPG, research updates, family support resources, and fundraising events.
  3. The Michael Mosier Defeat DIPG Foundation (defeatdipg.org): This foundation is dedicated to raising funds for DIPG research and advocating for increased awareness and funding. Their website offers information about DIPG, research updates, family support resources, and fundraising events.
  4. The ChadTough Foundation (chadtough.org): The ChadTough Foundation is committed to funding research and raising awareness for DIPG and other pediatric brain tumors. Their website provides information about DIPG, research updates, family support resources, and fundraising initiatives.
  5. Pediatric Brain Tumor Foundation (curethekids.org): The Pediatric Brain Tumor Foundation is a nonprofit organization that supports research, advocacy, and support for children with brain tumors, including DIPG. Their website offers resources, educational materials, support programs, and information about fundraising events.
  6. American Brain Tumor Association (abta.org): The American Brain Tumor Association provides information and resources for various types of brain tumors, including DIPG. Their website offers educational materials, support services, and updates on research advancements.
  7. Local Cancer Centers and Hospitals: Reach out to local cancer centers and hospitals with dedicated pediatric oncology departments. They can provide information about clinical trials, treatment options, and support services specific to your area.

Remember that each resource may offer different types of support and information, so exploring multiple sources can provide a comprehensive understanding of DIPG and available resources. It’s also important to consult with healthcare professionals who specialize in pediatric brain tumors for personalized advice and guidance.

Best Practices

When it comes to Diffuse Intrinsic Pontine Glioma (DIPG), a highly aggressive and challenging brain tumor, best practices involve a multidisciplinary approach aimed at providing the best possible care for the affected child. While DIPG has a poor prognosis and limited treatment options, the following best practices can help optimize patient care and support:

  1. Accurate Diagnosis: Prompt and accurate diagnosis is crucial for DIPG. Utilizing advanced imaging techniques, such as magnetic resonance imaging (MRI), with an experienced radiologist who is familiar with DIPG can aid in identifying characteristic features of the tumor.
  2. Multidisciplinary Care Team: Establishing a multidisciplinary team of healthcare professionals specializing in pediatric neuro-oncology is essential. This team typically includes pediatric neurosurgeons, neuro-oncologists, radiation oncologists, neuropathologists, and supportive care specialists. Collaboration among team members is vital for comprehensive care and decision-making.
  3. Palliative and Supportive Care: Given the aggressive nature of DIPG and limited treatment options, palliative and supportive care are critical components of the management plan. The focus should be on alleviating symptoms, optimizing the child’s quality of life, and providing emotional and psychological support for the child and their family.
  4. Radiation Therapy: Radiation therapy remains the primary treatment modality for DIPG. A high-dose focal radiation regimen, known as hypofractionated radiation therapy, is commonly employed to target the tumor while minimizing damage to surrounding healthy tissues. Treatment planning should involve experienced radiation oncologists who specialize in pediatric brain tumors.
  5. Clinical Trials and Innovative Therapies: Participating in clinical trials and exploring innovative therapies can offer potential avenues for new treatment options. Clinical trials may involve targeted therapies, immunotherapies, or other experimental approaches. Collaboration with research institutions and advocacy organizations can provide access to these opportunities.
  6. Shared Decision-Making: Open and transparent communication with the child’s family is crucial. Healthcare professionals should explain the prognosis, treatment options, potential benefits, and risks in a compassionate manner. Shared decision-making, taking into account the family’s values and preferences, helps ensure that treatment plans align with the child’s best interests.
  7. Psychosocial Support: Emotional and psychosocial support for the child and their family is essential throughout the DIPG journey. This includes access to counseling services, support groups, and resources that address the emotional, social, and practical challenges faced by the family.
  8. Collaboration and Research: Collaborating with national and international research networks is vital to advance our understanding of DIPG and develop new treatment strategies. Sharing data, participating in collaborative studies, and contributing to the research community can help drive progress in this field.

It’s important to note that DIPG remains a challenging disease, and best practices are continually evolving as new research emerges. Working closely with a specialized care team and seeking expert opinions can provide the most up-to-date and personalized care for children with DIPG.

Dr. Mueller’s best practices

NEW BEST PRACTICES TO KNOW UPON DIPG DIAGNOSIS

  • The most important first step is radiation to start relatively quickly (to preserve as much function as possible during the honeymoon period that many patients have, or to improve any functional loss before its permanent) -for some children with less symptoms this might be less critical
  • There is no documented difference between proton or photon radiation for DIPG/DMG, so whatever can be offered sooner should be considered. Radiation is standard of care used for many years and can generally be performed at most hospitals safely.
  • Steroids should be weaned as quickly and safely as possible-when and how much to use depends heavily on the clinical presentation and should be discussed with your team. Consider with your team whether it is necessary to start steroids proactively. Steroids do not inhibit the tumor from progressing. steroids help alleviate symptoms caused by inflammation surrounding the tumor. Learn the pros and cons of steroids early.
  • Biopsy should be performed by someone who has experience with the procedure. If a patient is critically ill and needs to start radiation therapy quickly, a biopsy can also be performed after completion of radiation therapy. Biopsies are necessary to confirm the diagnosis and determine the specific DIPG subtype. Having tissue available is becoming more and more important as many trials require information from the biopsy or actual tissue for enrollment. Be sure that the tissue is used for relevant testing-often the tissue is fairly limited. Data generated from the tissue can easily be shared with others.
  • Some clinical trials start WITH radiation, but most clinical trials start after radiation-all trials are listed on clinicaltrials.gov but be sure to discuss these options also with your team. It is challenging to sort through the different options alone.
  • Ask for a second opinion if you want to – it is highly recommended to get second opinions from different centers with experience treating DMG, as centers have different options to offer. Some trials are only available in certain places and providers have variable levels of comfort taking care of ALL diagnoses, including DIPG.
  • If your insurance is not covering all needs, talk to your social worker about secondary insurance options (ie. Medicaid).
  • Palliative care is often a helpful resource in the beginning to coordinate care, provide emotional support, etc. and palliative care can be activated WHILE on therapy in pediatric and plays a different role than classic “HOSPICE” end of life care in adults. Palliative care teams can really make your life much
    easier.
  • We will make more progress when working as a team-data sharing for a rare disease like DIPG is critical. One example is the DMG ACT. This group combines researchers around the globe to develop the best next therapies for children with DIPG. Anyone can join and we aim to make data available as soon as possible to enhance research further. Consenting to share data with the Children’s Brain Tumor Network allows researchers to learn from DIPG patients to drive treatments.

Dr. Sabine Mueller