0:00

So this is the patient with an expansile brainstem mass.

0:03

Within the pons, it engulfs the basilar artery.

0:06

That is one of the things that was historically said

0:08

as very classic for A-D-I-P-G.

0:13

Um, now there's some smaller

0:15

internal T two hyperintense cystic appearing areas, um,

0:20

and there's some peripheral enhancement along those, um,

0:24

T two hyperintense areas

0:26

and even some subtle susceptibility hypo intensity,

0:29

which probably goes with microscopic blood products.

0:34

Now, there's a heterogeneous appearance on diffusivity

0:39

on, on a DC maps,

0:41

and there's facilitated diffusion in these sort

0:44

of cystic appearing areas, which are presumably necrosis,

0:47

but it's heterogeneous.

0:49

Otherwise it's more hypo intense over here along the margin,

0:55

these margins of the, um,

0:57

these presumed necrosis, which would make sense.

1:00

So we use that to actually figure out a biopsy site.

1:03

Um, just to the left lateral aspect of these necrotic areas.

1:09

We didn't, the a areas anterior that are teach

1:13

that are very a DC hypo intense would be a great target if

1:17

you want to just say, oh, I want

1:19

to get the most cellular part of the lesion.

1:22

But the problem is, is that that's also

1:24

where the corticospinal tract fibers are.

1:26

So the biopsy was made.

1:28

We, we selected a site, um, more posteriorly.

1:33

Um, this was a diffuse midland glioma, the DIPG pattern,

1:37

the area of necrosis with the biopsy confirmed.

1:41

A WHO grade four diffuse midline glioma,

1:44

the H three K 27 M mutant, uh, with necrosis

1:48

and microvascular proliferation.

1:50

Well, all that is stuff that needs

1:54

to be confirmed on hi on biopsy,

1:57

but all the imaging characteristics were

2:00

pointing in that direction.

2:02

Now, those molecular changes have made it where a lot

2:07

of patients with DIPG, even if it's imaging

2:11

features are classic, they will still proceed to a biopsy.

2:14

Now, to get that molecular data,

2:16

which there may be targeted treatment, there may be,

2:19

you may find that it's actually a different entity

2:22

that responds differently.

Case: H3K27M Diffuse Midline Glioma With a DIPG Pattern, Grade 4

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0:00

So this is the patient with an expansile brainstem mass.

0:03

Within the pons, it engulfs the basilar artery.

0:06

That is one of the things that was historically said

0:08

as very classic for A-D-I-P-G.

0:13

Um, now there's some smaller

0:15

internal T two hyperintense cystic appearing areas, um,

0:20

and there's some peripheral enhancement along those, um,

0:24

T two hyperintense areas

0:26

and even some subtle susceptibility hypo intensity,

0:29

which probably goes with microscopic blood products.

0:34

Now, there's a heterogeneous appearance on diffusivity

0:39

on, on a DC maps,

0:41

and there's facilitated diffusion in these sort

0:44

of cystic appearing areas, which are presumably necrosis,

0:47

but it's heterogeneous.

0:49

Otherwise it's more hypo intense over here along the margin,

0:55

these margins of the, um,

0:57

these presumed necrosis, which would make sense.

1:00

So we use that to actually figure out a biopsy site.

1:03

Um, just to the left lateral aspect of these necrotic areas.

1:09

We didn't, the a areas anterior that are teach

1:13

that are very a DC hypo intense would be a great target if

1:17

you want to just say, oh, I want

1:19

to get the most cellular part of the lesion.

1:22

But the problem is, is that that's also

1:24

where the corticospinal tract fibers are.

1:26

So the biopsy was made.

1:28

We, we selected a site, um, more posteriorly.

1:33

Um, this was a diffuse midland glioma, the DIPG pattern,

1:37

the area of necrosis with the biopsy confirmed.

1:41

A WHO grade four diffuse midline glioma,

1:44

the H three K 27 M mutant, uh, with necrosis

1:48

and microvascular proliferation.

1:50

Well, all that is stuff that needs

1:54

to be confirmed on hi on biopsy,

1:57

but all the imaging characteristics were

2:00

pointing in that direction.

2:02

Now, those molecular changes have made it where a lot

2:07

of patients with DIPG, even if it's imaging

2:11

features are classic, they will still proceed to a biopsy.

2:14

Now, to get that molecular data,

2:16

which there may be targeted treatment, there may be,

2:19

you may find that it's actually a different entity

2:22

that responds differently.

Report

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Lesson

Introduction

Lesson

Meningiomas

Lesson

Non-glial Tumors

Lesson

2021 WHO CNS Tumor Classification

Lesson

Gliomas

Lesson

Pediatric Brain Tumor Cases

Case: H3K27M Diffuse Midline Glioma With a DIPG Pattern, Grade 4