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NF2: Manifestations of multiple CPA Meningiomas

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This is an MRI in an adolescent

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with neurofibromatosis type 2,

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and we can see enhancement

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in the internal auditory canals bilaterally.

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We also see sort of,

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in the right cerebellopontine angle cistern

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extending superiorly,

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this extra-axial enhancing lesion

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that has intermediate signal characteristics

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on T2 weighted imaging,

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is along the margin of the inferior

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aspect of the tentorium cerebelli.

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It also is relatively hyperintense and diffusion

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weighted imaging, and it has sort of an almost a

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dural tail-like appearance that we see on this.

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So, while this is in part in the right

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cerebellopontine angle cistern,

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this very well may represent a meningioma

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in the setting of neurofibromatosis type 2.

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While this is partially in the right

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cerebellopontine angle cistern,

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this has imaging characteristics that are suggestive

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of a possible meningioma, which is

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another lesion that is commonly seen in

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the setting of neurofibromatosis type 2.

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Notice the left vestibular schwannoma

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does not yet reach the porus acusticus.

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Therefore, there's no extension to

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the cerebellopontine angle cistern,

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and this fairly homogeneous enhancement.

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Now, this patient has other lesions.

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There's an enhancing lesion here,

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which is along the inferior aspect of the

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tentorium and adjacent to the junction

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of the sigmoid and transverse sinus.

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There's this lesion here down lower,

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which also looks like a meningioma.

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And then in the right lateral medullary cistern,

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we have another heterogeneous lesion that

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also very well could represent a meningioma

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with mass effect upon the brainstem.

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What else is it doing?

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Well, if we look on this T2 weighted image,

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we're seeing hyperintense signal

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in the right hemitongue.

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So, this is denervation atrophy.

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This is fatty changes in the right hemitongue

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related to involvement of and around the

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course of the right hypoglossal nerve,

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and into the right hypoglossal canal.

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Now, it's possible that there

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could be a hypoglossal schwannoma.

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But a lot of the other characteristics

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of this look like a meningioma with a

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dural tail that is not a morphology

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I would expect of a hypoglossal schwannoma.

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So, this patient has neurofibromatosis type 2

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with several meningiomas, including ones that

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are in the right cerebellopontine angle cistern,

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almost mimicking extension intracranially

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of a vestibular schwannoma,

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but likely representing a meningioma.

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And then also a lesion,

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likely a meningioma, impacting the

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right hypoglossal nerve, as evidenced by

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denervation atrophy in the right hemitongue.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Syndromes

Pediatrics

Neuroradiology

Neuro

Neoplastic

MRI

Congenital

Brain

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