0:00

De. Laser, we were talking von Hippel-Lindau

0:02

and that has led us to this case.

0:04

A patient that has a mass in the region of the vestibule

0:09

and the vestibular aqueduct.

0:12

And that might be hard to determine

0:13

cause the mass is pretty big.

0:14

Here is a postoperative study

0:16

from 3 months later,

0:17

where you can see they tried to resect it.

0:19

They couldn't get it out.

0:20

But let's scroll it on the T1,

0:24

and then let's scroll it over here on the T1 C+,

0:27

back and forth,

0:29

and when you look at it very carefully

0:31

my first impression is,

0:32

okay, those are flow voids in a glomus jugular tumor.

0:35

That would be overwhelmingly my first choice.

0:37

And then you look at it and you say,

0:38

"Well, wow. The jugular tubercle is still standing."

0:42

It's just kind of pushed over to the side.

0:44

It's almost like it's sloped a little bit.

0:46

And this lesion is not expanding or invading

0:50

the jugular bulb or the jugular vein.

0:52

It's just sitting right above it,

0:53

kind of floating there.

0:55

And then as you scroll up and down,

0:57

nowhere can you see the cochlea, the vestibule

1:02

and the other structures of the endolymphatic sac.

1:06

So, this is an endolymphatic sac tumor

1:08

and this is a cystadenoma.

1:10

Now there are 2 types of these.

1:11

There is the mixed-type,

1:13

which is a little less aggressive,

1:14

and then there's a papillary and adenomatous type

1:17

that invades the temporal bone.

1:18

I don't know which type this is,

1:19

although this one looks a little more aggressive.

1:22

It's pretty big,

1:23

it's sort of multifocal,

1:24

or at least it has a large contour to it

1:27

going back and forth along the cerebellum here,

1:30

but it doesn't actually invade the bone that we can see

1:32

on the MRI.

1:34

So again, I don't know the exact histology.

1:35

Most of these patients

1:36

present with hearing loss

1:39

and this patient does have hearing loss.

1:41

What percent of patients with von Hippel-Lindau get these?

1:44

Probably about 11% to 16% percent in the literature.

1:47

All right.

1:47

And what percent of patients with von Hippel-Lindau

1:50

will get some type of auditory vestibular symptoms?

1:53

Probably about 60%.

1:54

60%?

1:55

Yup.

1:55

And most of those

1:56

probably don't ever have a tumor identified?

1:58

Correct.

1:58

So probably, you have to screen those on a yearly basis

2:01

if they have hearing loss or vestibular symptoms,

2:03

like vertigo or tinnitus,

2:05

to make sure that they don't develop this

2:07

cause resection early is critical.

2:08

You can see we were unable to resect this.

2:11

Now, the differential diagnosis,

2:13

for me, for this specific lesion

2:15

would be glomus jugulare.

2:17

I would also include things like endolymphatic sac anomaly

2:20

in a different scenario.

2:22

Petrous apicitis,

2:23

things that effaced the petrous apex,

2:25

as this does on the left-hand side.

2:28

Meningioma of the petrous apex

2:30

metastasis is more aggressive,

2:32

and cholesterol granuloma or cyst,

2:33

a little more contained.

2:34

Bright on T1, bright on T2,

2:36

but in the petrous apex,

2:38

not eroding the petrous apex from the outside.

2:41

Pomeranz and Laser out.

2:43

Out.