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Dr. Laser,

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we've established a diagnosis of Von Hippel-Lindau

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in this 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.

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So it's been five years.

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And we've been following him,

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we looked at his cervical thoracic region,

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which is where hemangioblastoma likes 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 medullaris,

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I've actually not seen any in my experience

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in the cauda equina or in the tip of the filum.

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So now, we're scrolling through the lumbar,

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just for completeness,

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

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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.

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

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but let's look at its appearance

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on the axial T1-weighted image.

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And there is a cyst in the left kidney.

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Let's find that cyst.

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There's a cyst in the left kidney.

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It's quite a bit darker than this lesion.

1:02

Mm-hmm.

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So what could this thing be?

1:04

What are some options?

1:06

So, the association of renal cell carcinoma

1:10

is very high with Von Hippel-Lindau.

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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.

1:17

Now, there are multiple cysts throughout both kidneys.

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There's one there,

1:20

there's one there,

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and you do get cysts.

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In fact, you get cysts of the epididymis,

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although some people feel that they're epididymal cystadenomas

1:28

more consistently than they are epididymal cysts,

1:31

you also get cysts of the liver, the lungs,

1:33

the pancreas and of course, the kidneys.

1:36

But let's talk about some of the classic lesions

1:39

in Von Hippel-Lindau.

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Because you've got to screen

1:42

the entire individual for these lesions.

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And they include hemangioblastoma,

1:45

so the brain,

1:47

cervical thoracic region,

1:48

uncommon in the lumbar.

1:49

Then you've got retinal angiomas,

1:51

which are actually retinal baby hemangioblastomas.

1:55

We talked about the cysts,

1:57

including epididymal cysts.

1:58

You see a giant epididymal cyst...

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a giant epididymal cyst in a young man,

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you've got to think at least about Von Hippel-Lindau,

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especially if they've had neurologic symptoms.

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

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There are other renal manifestations we'll discuss in

2:14

another vignette: pheochromocytoma,

2:16

epididymal cystadenoma,

2:18

in addition to epididymal cysts.

2:20

In my world,

2:22

I actually distinguish the two

2:23

and I have seen both.

2:24

Proven.

2:25

So I separate them.

2:26

And then, endolymphatic sac tumor.

2:28

So one caveat,

2:29

you see an epididymal mass in a young man,

2:32

it's not automatically a cyst,

2:34

it could be a cyst or a cystadenoma.

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 the temporal bones.

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And the temporal bones,

2:44

especially the endolymphatic sac region.

2:47

Let's move on, shall we?

2:49

Let's.

2:49

Laser and P out.