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Complex NF2: Additional Lesions

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This is a patient with neurofibromatosis type 2

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that we separately discussed the evolution of

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vestibular schwannomas from

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this incidental finding in the left internal

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auditory canal to growing larger to very

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large and subsequent surgical resection.

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Now, beyond these vestibular schwannomas,

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there's a variety of other intracranial

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manifestations that are important to discuss.

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We've discussed that neurofibromatosis

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type 2 can also be thought of as MISME

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syndrome, multiple inherited schwannomas,

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meningiomas, and appendomas.

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Well, what does that look like?

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Well, if we look on this image here, in addition

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to these bilateral vestibular schwannomas, if

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we go posteriorly, we see this enhancing lesion

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off the inferior aspect of the cerebellum.

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That's a meningioma.

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If we scroll superiorly, we see

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this intraventricular lesion in the

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atrium of the left lateral ventricle.

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This is an intraventricular meningioma.

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If we go superiorly, you end up

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seeing multiple small lesions.

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This lesion right here is a meningioma.

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This lesion right here adjacent to the superior

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sagittal sinus near the vertex is a meningioma.

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There's multiple additional smaller ones.

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This one here is a meningioma that can

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be almost mistaken for a cortical vein,

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but this is actually a meningioma.

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So there's multiple additional findings.

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This here is a meningioma that was actually

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previously partially resected and is actually a

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partial extension into the left frontal sinus.

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So there's multiple different meningiomas,

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including this here around the superior

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sagittal sinus, the posterior third

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of the superior sagittal sinus.

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Which we can see best on this diffusion weighted

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imaging showing this near encasement of the

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superior sagittal sinus from this meningioma.

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And because the meningioma is so visible

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on diffusion-weighted imaging, there's a

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reasonable likelihood that it is an atypical

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or potentially high-grade meningioma.

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What else do we see in this patient?

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Well, here is a left

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oculomotor nerve schwannoma.

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Here is a focal protrusion along the

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lateral aspect of the left cavernous sinus.

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Which very well could be a meningioma

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with knowledge that the super lateral

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margin of the left cavernous sinus is

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where the oculomotor nerve enters, and

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immediately caudal to that is where the

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fourth nerve, the trochlear nerve enters.

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The abducens nerve or cranial nerve

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six goes through the cavernous sinus.

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But this seems to be separate from

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this oculomotor nerve schwannoma.

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And I do not see it fully filling

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and effacing the oculomotor cistern.

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So this very well could be a meningioma.

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So there's a variety of manifestations of this.

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Now this patient previously had had a lesion in

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the left temporal pole that had been resected.

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That was a rare manifestation known as meningio

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angiomatosis that involves and invaded the left

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temporal lobe and was the source of seizures.

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And so that is actually something that.

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Initially, what brought this patient to

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medical attention, and it was only later that

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the vestibular schwannomas were identified.

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So there's a wide variety

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of intracranial findings.

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Now, as I mentioned, there's the term, multiple

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inherited schwannomas, which we have the very

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least these two large bilateral vestibular

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schwannomas and the left oculomotor schwannoma.

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meningiomas, we saw multiple meningiomas,

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including this intraventricular

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meningioma, and this Tentorial meningioma.

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But the other part of MISB

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syndrome is appendomoma.

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And if we look at this spine imaging, we

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see a focal enlargement of the mid to upper

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cervical cord with central T2 hyperintense

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signal and post-contrast enhancement.

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In the setting of neurofibromatosis type 2, this

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is consistent with a spinal cord ependymoma.

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Now on this, we also see this large lesion.

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Intradural extramedullary that

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extends through and expands this

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neural foramen, this is a schwannoma.

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Now, in these patients we often will

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see other little enhancing lesions.

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So there's multiple additional

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intradural enhancing lesions.

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Those are likely all small schwannomas.

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Now, because this is a genetic

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defect in neurofibromatosis type 2,

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a genetic abnormality, these are not

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considered to be metastatic deposits.

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This is considered to be multicentric disease.

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Multicentric disease means each one of these

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lesions is a separately derived tumor that came

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about because of the germline mutation within

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chromosome 22 for neurofibromatosis type 2.

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So this patient has all the characteristic

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findings of neurofibromatosis type 2.

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We have multiple schwannomas,

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both intracranial and spinal.

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We have multiple meningiomas, and

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we have a spinal cord ependymoma.

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This shows that even though neurofibromatosis

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type 2 is not the most common disease

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process in the general population, These

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patients end up having frequent contact

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with the healthcare system, frequent medical

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imaging, and so it is not uncommon to

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see follow-up studies on these patients.

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I have several neurofibromatosis type 2

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patients that between brain, spine, and various

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other body parts have well over 50 to 100.

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different MR examinations in their past.

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So it's very common to see imaging on these,

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even more common than you would expect based

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upon the disease prevalence in the population.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Syndromes

Pediatrics

Neuroradiology

Neuro

Neoplastic

Musculoskeletal (MSK)

MRI

Congenital

Brain

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