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H3K27 Glioma

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You here is a 15-year-old female patient.

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who presented with headaches.

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And this is the baseline study which showed a

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tumor in the left thalamus, as you can see,

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causing slight mass effect on the

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posterior third ventricle.

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The tumor does show a little bit of

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areas of patchy enhancement,

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and also has some increased cellularity as

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seen on the diffusion scans over here.

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As I mentioned earlier,

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majority of these tumors in either midline or

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para midline location, in a younger patient,

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we should be worried about H3K27

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mutant diffuse midline gliomas.

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And this is what happens to this patient.

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Within six weeks,

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the follow-up MRI shows that the tumor actually is

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increasing rather quickly.

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Within six weeks,

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the tumor has increased in size.

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There are new areas of enhancement developing in this tumor.

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At this time,

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the patient underwent surgery and was proven

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to be H3K27 mutant glioma.

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The important thing in these cases, as we all know now,

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that these tumors are very aggressive tumors

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and they can progress rather quickly.

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And this is what happens classically to these patients.

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This is a scan done a year later.

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So one-year follow-up and you can see the tumor is

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actually now spreading through the left meningeal route.

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There is spread within the ventricle,

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there is subependymal tumor spread, multifocal disease.

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And this is, again, a bad tumor and has a poor prognosis.

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This unfortunate 15-year-old girl ended up dying from

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this tumor within 15 months after the initial diagnosis,

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as expected for these very aggressive tumors.

Report

Description

Faculty

Rajan Jain, MD

Professor of Radiology and Neurosurgery

New York University Grossman School of Medicine

Tags

Pediatrics

Oncologic Imaging

Neuroradiology

Neoplastic

MRI

Brain

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