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Case: H3K27M Midline Glioma, Grade 4

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Alright, let's shift now from some of the adult

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gliomas where we separated things into astrocytomas

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and the groden gliomas.

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And now let's look at some of the pediatric tumors

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that we described with regard to some of the mutations.

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So this is a patient who has an H three

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K 27 M mutation and

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therefore is one of those diffuse midline gliomas.

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And this is a typical appearance of them in which you see

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that on the sagittal T one Wade scan, you have a mass

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that is either in the third ventricle or the thalamus,

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but in any case, it's in the midline.

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Here's our pons and medulla.

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You notice that the cerebellar tonsils are being pushed

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downward here to the frame

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and magnum on post gadolinium hand scans.

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This lesion is again growing

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into the third ventricle,

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but probably expanding out into the thalamus.

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We have areas here of enhancement, which I would expect

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would not be part

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of the third ventricle and more superiorly.

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You can see the third ventricle is a little bit displaced

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to the right side by this paramedian left-sided component.

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On the T two eight scan, we have a little bit

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of a motion blur, but what we readily

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identify is the presence of trans penal CSF flow

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or interstitial edema suggesting acute hydrocephalus

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because of the obstruction at the third ventricle

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and aqueduct, uh, you notice

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that this tumor has a intimate association

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with the internal cerebral veins.

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Coming back to the vein of galin, an important point

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for the surgeons, again, do not want to

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occlude these vessels or cauterize these vessels

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because of the potential for a venous infarction.

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On this case, we have the A DC map

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and there are two different boxes

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that have been measured here.

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One with a mean of 715, the other with a mean

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of 775 indicating that this is likely a higher grade tumor.

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Remember that the H three K 27 M

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and the H 3G 34 M tumors are both

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grade four high grade

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gliomas occurring in the pediatric population.

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So I think this is a pretty nice example of

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the imaging features of what one would expect

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with these midline pediatric gliomas.

Report

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Oncologic Imaging

Neuroradiology

Neoplastic

MRI

Brain