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NF1 with High Grade Glioma

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This is an MRI of the brain

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in an eight-year-old child

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with neurofibromatosis type 1.

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We see areas of myelin vacuolization

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in the globus pallidus bilaterally and

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in the left cerebral peduncle,

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in the left ventral pons,

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in the region of the dentate nucleus,

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and deep cerebellar gray and white matter

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bilaterally, left greater than right.

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We can see, actually,

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an expansile area in the left posterior

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aspect of the medulla oblongata

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Now, we don't see any abnormal enhancement in

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that area of expansile involvement

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in the medulla oblongata,

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but it's definitely something to

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be aware of and keep an eye on.

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But the most important thing to notice

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in this patient is this focal lesion here.

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This is in the parasagittal aspect

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of the left frontal lobe.

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Heterogeneously hyperintense

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on T2-weighted imaging with areas of actual

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relative T2 hypointense signal.

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It's relatively homogeneously hypointense

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on T1 weighted imaging

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and demonstrates heterogeneous

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post-contrast enhancement.

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If I look on diffusion-weighted imaging,

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I see hyperintense signal

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that the ADC maps.

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The ADC maps confirm as being relatively

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reduced water diffusion with

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an average ADC of 940.

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Now, generally speaking,

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while not an exact cutoff below 1000 for

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the ADC is often associated

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with high-grade gliomas.

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So, this patient with neurofibromatosis type 1

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has this focal lesion with

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surrounding mass effect and edema,

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heterogeneous enhancement,

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and reduced water diffusion.

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At biopsy,

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this was confirmed to be a high-grade glioma.

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So this is the patient with

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neurofibromatosis type 1,

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who developed a high-grade glioma.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Syndromes

Pediatrics

Neuroradiology

Neuro

Neoplastic

MRI

Brain

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