01What Is Diffuse Midline Glioma?

Diffuse midline glioma, H3 K27-altered, is the WHO classification for a family of tumors that arise along the brain's midline, most often in the pons, the part of the brainstem that gives this tumor its older name, DIPG. These tumors are defined less by where exactly they sit than by how they grow: instead of forming a single mass with a clear edge, the tumor cells spread through and mix with the normal fibers of the brainstem. That growth pattern, not the tumor's grade in the traditional sense, is what makes it so difficult to treat.

It's a disease of childhood far more often than of adulthood, and the pons is involved in the large majority of cases. Because the pons controls basic functions like eye movement, facial sensation, swallowing, and motor pathways to the limbs, even a tumor of modest size can produce significant symptoms early on.

02Where It Occurs

PonsBy far the most common site, giving the tumor its classic name.
ThalamusA less common location, with its own pattern of neurological signs.
Spinal CordUncommon; the same biology can occur along the midline of the spine.

03Symptoms

Because the pons is packed with cranial nerve nuclei and the major pathways connecting the brain to the rest of the body, symptoms tend to appear over just weeks rather than months, and often come as a cluster rather than one isolated complaint. Families frequently describe:

  • Double vision or a drooping, asymmetric face
  • An unsteady, wide-based walk or general clumsiness
  • Weakness on one side of the body
  • Trouble swallowing or a change in the voice
  • Morning headaches with vomiting, from pressure building up behind the tumor

04Diagnosis

In most children, the MRI appearance alone, a tumor expanding the pons diffusely rather than sitting within it, is distinctive enough that a confident diagnosis can be made without tissue. Even so, we routinely add perfusion imaging, spectroscopy, and diffusion tensor imaging (DTI). Beyond confirming what the standard sequences already suggest, these techniques map the exact position of critical fiber tracts, which is what allows a biopsy needle, or occasionally a resection, to be planned along the safest possible corridor.

05How We Approach Treatment

Radiotherapy is the backbone of treatment, and for most patients it remains the single most effective way to shrink the tumor and relieve symptoms, sometimes dramatically, for a period of months. What has changed in recent years is what happens around it.

We now take a stereotactic needle biopsy in essentially every patient before radiotherapy begins. Removing the whole tumor isn't possible given how it grows, but a small tissue sample is enough to run a full molecular workup, including next-generation sequencing, alongside standard pathology. That workup doesn't just confirm the diagnosis; it identifies specific mutations that, in a subset of patients, open the door to a targeted drug or a clinical trial that wouldn't otherwise be an option, and it gives us something concrete to fall back on if the tumor progresses and a second round of treatment is needed.

Surgical resection is the exception rather than the rule. A small number of these tumors grow with an exophytic component, a portion that bulges outward from the brainstem rather than infiltrating it, and in that specific setting partial or complete removal can be safely attempted. For the majority of patients, though, the plan is biopsy, radiotherapy, and molecular-guided therapy where the biology allows it.

Treatment Roadmap

How We Approach It

Here's how we think through the decision for DIPG, and what to expect from biopsy through radiotherapy and beyond.

Imaging Review

MRI, together with perfusion, spectroscopy, and DTI sequences, establishes the diagnosis and maps the safest corridor through the brainstem.

Tumor Board Discussion

Neurosurgery, pediatric neuro-oncology, radiation oncology, and radiology review the imaging together and agree on a biopsy or, in select cases, a resection.

Which Path Fits This Tumor

Almost every patient follows the same core path; a small minority are candidates for something more. Tap either one below.

A stereotactic needle biopsy under neuronavigation confirms the diagnosis and provides tissue for a full molecular workup, including next-generation sequencing. Radiotherapy starts as soon as pathology is back, and if the molecular profile points to a targeted option, that gets folded into the plan alongside it.
When part of the tumor bulges outward from the brainstem rather than growing through it, that portion can sometimes be safely removed, partially or completely. Radiotherapy and molecular testing still follow, exactly as they would otherwise.
After Radiotherapy

Once radiotherapy finishes, we start any targeted or chemotherapy agents the molecular results support, and the tumor is followed with regular imaging. The molecular data stays on file, and it becomes relevant again if further treatment is ever needed.

Before Workup

Full MRI protocol, anesthesia clearance, and a joint plan between neurosurgery and radiation oncology for how the biopsy and treatment will proceed.

Day 0 The Biopsy

Done under general anesthesia through a needle-sized opening, guided in real time by neuronavigation. There's no large incision to recover from.

Day 1 Going Home

A neurological check the following morning is usually all that stands between the biopsy and discharge.

About a Week Results Come Back

Pathology and the molecular panel are finalized, and radiotherapy is scheduled to begin.

The Following Weeks Radiotherapy

Daily sessions over several weeks on our adaptive LINAC platform, with any indicated drug therapy layered in once the molecular results are complete, and imaging follow-up continuing afterward.

Shown for a typical case. Individual course may vary.