0:00

Here is a patient who is being evaluated

0:03

for left-sided hearing loss.

0:05

I'm going to show you the T1-weighted scan before

0:09

contrast. So here on the T1-weighted scan,

0:12

as we come from the lower temporal bone,

0:15

we come into a lesion that has high

0:17

signal intensity, pre-contrast,

0:22

with little locules of bright signal intensity,

0:25

as well as intermediate signal intensity.

0:28

And this lesion is oriented along the plane

0:31

of the petrous bone and the petrous apex.

0:35

So this is what we would expect to see in a patient

0:38

who has an endolymphatic sac tumor.

0:40

What would I want to see on T2-weighted scan?

0:42

I'd like to see it be heterogeneous,

0:44

and I'd like to see it show contrast enhancement.

0:47

So here in our post-gad scan,

0:50

you see that the lesion shows

0:52

a more uniform enhancement.

0:57

And it looks like there may be even some calcified

0:59

matrix to this lesion by virtue of the persistent

1:02

low signal intensity on this post-contrast scan.

1:07

How about on T2-weighted?

1:10

This is the CISS image,

1:13

and as we scroll up,

1:18

you can see that it does indeed have heterogeneous

1:21

signal intensity with both bright areas

1:23

as well as dark areas.

1:25

Now, I ask, as I scroll through this case

1:28

and through the lesion,

1:30

how many of you are able to pick

1:32

up any additional findings?

1:36

Well,

1:38

hopefully you saw that there was a contrast

1:41

enhancing round mass in the periphery of the

1:45

left cerebellar hemisphere. And in fact,

1:50

an additional smaller mass

1:53

in the anterior portion of the

1:55

right cerebellar hemisphere.

1:58

So given these additional findings,

2:02

we would suggest that this patient has an

2:05

endolymphatic sac tumor associated with von

2:08

Hippel-Lindau disease with cerebellar

2:11

hemangioblastomas. So once I put that into my head,

2:16

I think, ah,

2:17

but I know that with von Hippel-Lindau disease, there

2:21

is a 30% rate of potential bilaterality

2:25

of my endolymphatic sac tumor.

2:27

So I better go back and double-check my other petrous

2:30

bone. Oh, it looks clean, so I think I'm fine.

2:34

Remember also that von Hippel-Lindau disease is

2:39

associated with retinal hemangioblastomas also.

2:43

So now I'm going to shift this case downward and

2:48

make sure that I look with high conspicuity at the

2:52

globes to see whether any areas of asymmetric

2:56

enhancement. And what I notice is that this area

3:00

of the retinal layer seems to be a little bit

3:05

thicker than the contralateral

3:07

side at the same levels.

3:09

And this patient indeed had a retinal

3:12

hemangioblastoma on the right side, as well.

3:15

The next thing to do, scan the abdomen,

3:18

because patients who have von Hippel-Lindau disease

3:20

have a higher rate of renal cell carcinoma,

3:22

pancreatic tumors, pancreatic cysts, etc.

3:26

But this is a nice example of a patient whose

3:28

initial presentation was for the endolymphatic

3:31

sac tumor, and through that,

3:33

we found additional findings that clinched

3:35

the diagnosis of von Hippel-Lindau disease.

3:38

I told you I love this diagnosis.