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Meningiomatosis

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0:00

Dr, Laser,

0:01

this 59-year-old woman,

0:03

has multiple abnormalities.

0:06

We have an axial T2 on the left,

0:07

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

0:11

a susceptibility-type sequence on the right,

0:15

and immediately we see 2 large bulky,

0:17

almost dumbbell-shaped masses

0:19

and

0:20

they clearly are extra-axial,

0:22

don't you think?

0:23

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

0:25

on the right aspect of the lesion,

0:28

and then you also have some vasogenic edema around it

0:30

and it clearly buckles the brain

0:34

towards the right.

0:35

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

0:38

for extra-axial lesions like meningiomas,

0:40

although there are some

0:41

extra-axial lesions like metastasis and melanomas

0:45

dural melanomas that could give you that too,

0:47

but the take-home message is,

0:49

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

0:51

is perfectly compatible with meningioma.

0:54

And then we keep scrolling around

0:56

and the gradient echo can sometimes be really helpful,

0:59

especially if the meninges are calcified

1:01

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

1:05

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

1:11

and as we get into the falcotentorial region,

1:13

near the top,

1:14

here's another one.

1:15

It sticks out very nicely because of its

1:17

calcific character,

1:19

and there, it continues on as a

1:22

sessile posterior parafalcine meningioma,

1:25

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

1:29

And then finally, oh my goodness,

1:31

look at this.

1:31

We've got another big one here,

1:33

another one here

1:33

another one here,

1:34

and possibly even another one there,

1:36

and then a few more.

1:37

So this patient has,

1:38

you know,

1:39

8, 9, 10, 11, 12 meningiomas,

1:42

which isn't usual,

1:43

and we have talked previously about the entity of

1:46

phacomatotic meningiomatosis.

1:49

We said it's a familial condition.

1:51

It tends to be unilateral,

1:53

tends to affect one side but can be bilateral,

1:56

and it is caused by a mutation

1:58

along the germ line of SMARCB1 and SMARS1.

2:03

There are also some syndromic

2:05

meningioma syndromes that include

2:08

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

2:11

BAP1 or a tumor predisposition syndrome,

2:15

multiple endocrine adenomatosis type 1 or MEA1,

2:18

and basal cell nevus syndrome,

2:20

which really is kind of Cowden syndrome.

2:24

So now, let's move on

2:26

to the contrast-enhanced portion of the study.

2:29

We've got the T1 without contrast,

2:31

the T1 with contrast,

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and a coronal with contrast that shows the full

2:37

extent of these lesions.

2:40

And you can see that the left transverse sinus is

2:44

very compressed.

2:46

So, it's probably not occluded,

2:47

but it's on its way to being secondary

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occluded due to slow flow and compression.

2:52

The patient does not have a venous infarct.

2:53

We discussed before that there is

2:56

slow compression that occurs

2:58

that allows collaterals to develop.

3:00

So, rarely do you see venous infarcts associated

3:03

with dural-based meningiomas, even if they affect

3:06

the dural venous sinus,

3:07

but you absolutely want to look there.

3:10

What are some other take-home messages

3:12

about this case where we have

3:14

so many meningiomas present?

3:15

We talked about vasogenic edema,

3:17

you pointed out the cleft sign,

3:19

the CSF cleft sign on the T2-weighted image,

3:23

the dural-based nature of the lesion,

3:24

the multiplicity of lesion,

3:26

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,

3:33

one thing to always consider is

3:36

mass effect inside the posterior fossa,

3:38

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

3:41

obstructive hydrocephalus is always is a possibility.

3:43

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?

3:50

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,

3:56

an important clinical point,

3:57

and one that is relevant in this case.

3:59

These temporal horns are too big.

4:01

Mm-hmm.

4:01

And the ventricles are a little big,

4:03

and the cerebellar tonsils are a little saggy.

4:06

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

4:09

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

4:13

group of meningiomas.

4:14

So this patient, unfortunately,

4:16

something you often overlook,

4:17

unfortunately, is developing obstructive hydrocephalus.

4:21

And while right now, at this very moment,

4:22

it's not a medical emergency.

4:23

It could be pretty shortly,

4:25

so this is absolutely a phone call.

4:26

Great pickup.

4:27

Let's move on, shall we?

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Pediatrics

Neuroradiology

Neoplastic

MRI

Brain

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