0:00

Douglas Azar.

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We, we've established a diagnosis of Von Hippel in this

0:05

now 30 something year old man.

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When we first saw him in one of the earlier vignettes,

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it was 2012, so it's been five years

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and we've been following him.

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We, we looked at his cervical thoracic region, which is

0:16

where Heman Neoblastoma is like to live.

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They don't like to live in the lumbar region,

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although I have seen a few of them in the conus meis.

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I've actually not seen any in my experience in the, in the,

0:26

uh, called aquina or in the tip of the file.

0:30

So now we're scrolling through the lumbar just

0:32

for completeness to make sure we don't have any down below.

0:36

And as we scroll the axial projection

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and then the sagittal projection,

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one thing really sticks out in the sagittal projection,

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which is this bright lesion.

0:45

And most of the time we would attribute that to a cyst.

0:47

But let's look at its appearance

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on the axial T one weighted image.

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And there is a cyst in the left kidney.

0:54

Let's find that cyst. There's a cyst in the left kidney.

0:59

It's quite a bit darker than this lesion. Mm-Hmm.

1:03

So what could this thing be? What are some options?

1:06

So the association

1:08

of renal cell carcinoma is very high with Von Hippel Lindel.

1:11

So that would be the number one consideration

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when you see something like this.

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So a cystic renal cell has to be excluded.

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Now there are multiple cysts throughout both kidneys.

1:19

There's one there, there's one there. And you do get cysts.

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In fact, you get cysts of the epi demus,

1:25

although some people feel that they're epidermal cyst

1:27

adenomas more consistently than they are epidermal cysts.

1:31

You also get cysts of the liver, the lungs, the pancreas,

1:34

and of course the kidneys.

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But let's talk about some

1:37

of the classic lesions in von Hippo Lindo.

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'cause you've gotta screen the entire

1:42

individual for these lesions.

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And they include heman glioblastomas of the brain,

1:46

cervical thoracic region, uncommon in the lumbar.

1:49

Then you've got retinal angios, which are actually

1:52

retinal baby heman neuroblastomas.

1:55

We talked about the cysts, including epidermal cysts.

1:58

You see a giant epidermal cysts,

2:00

a giant epidermal cyst in a young man.

2:02

You've gotta think at least about von hip, Belinda,

2:05

especially if they've had neurologic uh, symptoms.

2:08

Then you've got, uh, renal cysts and renal cell carcinoma.

2:11

Uh, there are other renal manifestations we'll discuss in

2:14

another vignette, pheochromocytoma,

2:16

epidermal cystadenoma in addition to epidermal cysts.

2:21

In my world, I actually distinguish the two

2:23

and I have seen both proven, so I separate them.

2:26

And then endo lymphatic sac tumor.

2:28

So one caveat you see at epidermal mass in a young man,

2:32

it's not automatically a cyst.

2:34

It could be a cyst or a cyst. Adenoma.

2:37

Don't forget to look at the triad in the brain,

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which is the globes.

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The cerebellum and

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The temporal bones. And

2:43

the temporal bones, especially the

2:46

endo lymphatic sac region.

2:48

And we have come down into the lumbar region

2:50

to continue on with his screening.

2:53

And on the lumbar study, they found several renal masses.

2:56

I'm not gonna show you the sagittal,

2:58

but the axial shows multiple light bulb hyperintense masses

3:02

in the left kidney, and smaller ones in the right kidney.

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Let's scroll a little bit so you can see them all.

3:07

Notice this one

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does not have the typical simple water signal like

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CSF like this one.

3:14

This one's closer to CSF. This one is a bit more gray.

3:18

I think the same can be said for this lesion here. Mm-Hmm.

3:22

It's, it's not quite as bright as some of the other cysts.

3:25

And when you look at the T one, that's

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where the information really shines.

3:29

That doesn't look anything like CSF doesn't

3:31

look anything like a simple cyst.

3:33

We go to the opposite side.

3:34

Let's take a look at some of these lesions over

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here and scroll them.

3:38

And that one looks more like a cyst. It's closer to CSF.

3:41

It's nice and bright here. That one, not so much. Mm-Hmm.

3:44

It's a little gray. It's brighter than the cortex

3:47

of the re of the kidney.

3:48

And it's not as bright as the cerebral spinal fluid.

3:51

So these lesions require further investigation.

3:54

How should we investigate them?

3:56

So you'd wanna do a contrast enhanced pre

3:58

and post MR of the abdomen,

4:00

specifically mentioning the kidneys

4:02

with renal mass protocol.

4:04

And, and I, when I, when I do these dynamic studies

4:06

and I do like to do them

4:07

dynamically, I like to subtract them.

4:09

So I really get a beat on whether

4:11

there's any enhancement at all.

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'cause a cyst should have the thinnest rim

4:15

of enhancement or none at all.

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You should see no nodularity, no papillary projections,

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and certainly no solid enhancement.

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And you've gotta look for that very subtle nodularity.

4:24

Now the things you're looking for in Von Hipow include not

4:27

just cysts and clear cell carcinoma,

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which occurs somewhere in the range of 30% of individuals,

4:33

15 to 50% cyst, 25 to 63, 60 3%,

4:38

but also angios, which we often overlook 7% of the time.

4:42

Yes, you get renal angios.

4:43

This is who gets them VHL patients

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and adenomas of the kidney.

4:48

14%. So if it's solid and round

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and it enhances, it doesn't automatically mean

4:53

it's a renal cell carcinoma.

4:55

It could be an adenoma.

4:56

And frequently these, these cancers are multifocal.

5:00

They're not, they're not just unifocal.

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So you could have them in both kidneys,

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you could have multiple ones in one kidney

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and frequently will will act to remove these

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with localized conservative therapy rather

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than take the whole kidney out.

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Otherwise you'll end up taking out both kidneys

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and they'll end up with a transplant, which is a bad idea.

5:17

Yep. Pom ran are out.