What is the definition of Diffuse Midline Glioma H3 K27M-Mutant?

Diffuse intrinsic pontine glioma (DIPG) is a type of aggressive brain tumor that occurs in the pons, part of the brainstem. The pons is important for many bodily functions, including regulating breathing, heart rate, bladder control, balance, and more.  Diffuse intrinsic pontine glioma primarily affects children, with roughly 150-300 new diagnoses per year. 

What are the causes for Diffuse Midline Glioma H3 K27M-Mutant?

The cause of diffuse intrinsic pontine glioma is not currently known. Research is being done to identify if certain genetic mutations may be causing DIPG. 

What are the symptoms for Diffuse Midline Glioma H3 K27M-Mutant?

Symptoms of diffuse intrinsic pontine glioma often develop quickly. Symptoms may include poor coordination, arm and leg weakness, as well as difficulty controlling eye movements and facial expressions. Some individuals may experience difficulty speaking or eating as well. 

What are the current treatments for Diffuse Midline Glioma H3 K27M-Mutant?

The main treatments for diffuse intrinsic pontine glioma currently include radiation therapy and experimental chemotherapy.  Radiation therapy is generally used for newly diagnosed DIPG. Radiation therapy involves the use of high-energy beams, including X-rays or protons, to destroy tumor cells. Various clinical trials have shown that routine chemotherapy is not effective for treating DIPG. Newer treatments combine chemotherapy with biologic therapy that directly target the tumor.  Because of the tumor's location in the brainstem, it is rarely possible to perform surgery without risk of causing neurological damage. 

What is the outlook (prognosis) for Diffuse Midline Glioma H3 K27M-Mutant?

The prognosis for diffuse intrinsic pontine glioma is very poor. Although there is no cure, a small percentage of patients survive.
Clinical Trial
  • Status: Recruiting
  • Phase: Phase 1
  • Intervention Type: Biological
  • Participants: 32
  • Start Date: December 12, 2018
Phase I REsearch on Multi-antigen T Cell Infusion Against Neuro-oncologic Disease
Clinical Trial
  • Status: Active, not recruiting
  • Phase: Phase 1
  • Intervention Type: Drug, Other, Radiation
  • Participants: 6
  • Start Date: October 5, 2015
A Phase I Study of Suberoylanilide Hydroxamic Acid (SAHA, Vorinostat) With Temsirolimus in Children With Newly Diagnosed or Progressive Diffuse Intrinsic Pontine Glioma (DIPG)