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Case: Meningiomatosis

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0:00

Dr, Laser,

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this 59-year-old woman,

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has multiple abnormalities.

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We have an axial T2 on the left,

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a FLAIR in the middle, and a GRE or a gradient echo,

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a susceptibility-type sequence on the right,

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and immediately we see 2 large bulky,

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almost dumbbell-shaped masses

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and

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they clearly are extra-axial,

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don't you think?

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Correct. You have a CSF cleft sign on the left,

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on the right aspect of the lesion,

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and then you also have some vasogenic edema around it

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and it clearly buckles the brain

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towards the right.

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So, I mean the vasogenic edema doesn't bother you at all

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for extra-axial lesions like meningiomas,

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although there are some

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extra-axial lesions like metastasis and melanomas

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dural melanomas that could give you that too,

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but the take-home message is,

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the fact that there's a fair amount of vasogenic edema here

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is perfectly compatible with meningioma.

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And then we keep scrolling around

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and the gradient echo can sometimes be really helpful,

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especially if the meninges are calcified

1:01

and here we've got one and 2 easily seen,

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correlating very nicely with the FLAIR and the T2,

1:08

but let's keep going, shall we?

1:09

Let's go up a little bit higher

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and as we get into the falcotentorial region,

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near the top,

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here's another one.

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It sticks out very nicely because of its

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calcific character,

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and there, it continues on as a

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sessile posterior parafalcine meningioma,

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and there is yet another one along the left-sided convexity.

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And then finally, oh my goodness,

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look at this.

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We've got another big one here,

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another one here

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another one here,

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and possibly even another one there,

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and then a few more.

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So this patient has,

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you know,

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8, 9, 10, 11, 12 meningiomas,

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which isn't usual,

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and we have talked previously about the entity of

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phacomatotic meningiomatosis.

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We said it's a familial condition.

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It tends to be unilateral,

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tends to affect one side but can be bilateral,

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and it is caused by a mutation

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along the germ line of SMARCB1 and SMARS1.

2:03

There are also some syndromic

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meningioma syndromes that include

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Cowden's disease, Werner syndrome, Rubinstein-Taybi,

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BAP1 or a tumor predisposition syndrome,

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multiple endocrine adenomatosis type 1 or MEA1,

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and basal cell nevus syndrome,

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which really is kind of Cowden syndrome.

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So now, let's move on

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to the contrast-enhanced portion of the study.

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We've got the T1 without contrast,

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the T1 with contrast,

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and a coronal with contrast that shows the full

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extent of these lesions.

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And you can see that the left transverse sinus is

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very compressed.

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So, it's probably not occluded,

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but it's on its way to being secondary

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occluded due to slow flow and compression.

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The patient does not have a venous infarct.

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We discussed before that there is

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slow compression that occurs

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that allows collaterals to develop.

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So, rarely do you see venous infarcts associated

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with dural-based meningiomas, even if they affect

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the dural venous sinus,

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but you absolutely want to look there.

3:10

What are some other take-home messages

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about this case where we have

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so many meningiomas present?

3:15

We talked about vasogenic edema,

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you pointed out the cleft sign,

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the CSF cleft sign on the T2-weighted image,

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the dural-based nature of the lesion,

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the multiplicity of lesion,

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the compression of the dural venous sinuses.

3:28

Anything else you think we should add?

3:31

No, just when you have lesions in the posterior fossa,

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one thing to always consider is

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mass effect inside the posterior fossa,

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like a rigid area where if you have anything that's extra,

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obstructive hydrocephalus is always is a possibility.

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So, that's an important take-home point to definitely comment on.

3:48

I think that's a great point, and you know what?

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I'm not sure there isn't the level of obstruction here.

3:53

I mean you brought up

3:55

not just a great point,

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an important clinical point,

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and one that is relevant in this case.

3:59

These temporal horns are too big.

4:01

Mm-hmm.

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And the ventricles are a little big,

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and the cerebellar tonsils are a little saggy.

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They're low lying and they're a little bit pointy,

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and that is, in all likely, related to this posterior fossa

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group of meningiomas.

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So this patient, unfortunately,

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something you often overlook,

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unfortunately, is developing obstructive hydrocephalus.

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And while right now, at this very moment,

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it's not a medical emergency.

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It could be pretty shortly,

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so this is absolutely a phone call.

4:26

Great pickup.

4:27

Let's move on, shall we?

Report

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Oncologic Imaging

Neuroradiology

Neoplastic

MRI

Brain

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