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Case: 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

Faculty

Rajan Jain, MD

Professor of Radiology and Neurosurgery

New York University Grossman School of Medicine

Tags

Oncologic Imaging

Neuroradiology

Neoplastic

MRI

Brain

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