0:01

So in this case, I'm going to show,

0:03

share with you T2-weighted image. A 55-year-old female,

0:07

and T1.

0:10

So let's do the T2-weighted image.

0:16

So a lot going on in the pancreas here,

0:21

and it may be of some utility to look at some other organs

0:24

in this instance.

0:27

Particularly the kidneys and maybe adrenal glands, as well.

0:30

I'll give you the T1-weighted images.

0:35

So no real hyperintense...

0:37

T1 hyperintense content in some of the stuff that we're looking at.

0:39

Then the post-contrast sequences to the pancreas,

0:42

little bit of motion here.

0:44

Apologize for that.

0:46

Again, look at the other organs as well.

0:48

A lot of stuff going on the others...

0:49

some of the other organs.

0:55

This is less motion here, so this may be easier for you to look at.

1:01

Pancreas looks certainly very interesting, but some of the other organs do, as well.

1:07

Okay, let's post our last question for the hour.

1:11

So in this instance, I'm really asking the group to

1:16

come up with a diagnosis of what they think this patient has.

1:19

Based on that, asking you to maybe take it a step further.

1:22

What's the most common CNS tumor seen in the disease that this patient has?

1:26

Is it a Hemangioblastoma?

1:28

Is it a Neurofibroma?

1:29

Is it a GBM or a Subependymal giant cell astrocytoma?

1:35

Known as SEGA tumors, I believe.

1:37

I'm certainly no neurologist.

1:38

So if there's a neurologist out there

1:40

on the call, you know, you feel free to educate me

1:44

a little bit if I'm incorrect about any of these things.

1:47

Yes. So Hemangioblastoma.

1:48

So it was the most...

1:49

It was the correct answer, most people get.

1:51

So what am I showing you here?

1:53

So you're seeing a pancreas that is replaced by numerous, numerous cysts.

1:58

And when you see that appearance, you got to think of Von Hippel-Lindau disease.

2:01

Now, it's a rare disease.

2:03

We see it in a select few patients.

2:06

It is hereditary, autosomal dominant,

2:08

and it affects multiple organs, resulting in multiple neoplasms.

2:12

Now in the pancreas, you're going to see a bunch of cysts.

2:14

Now, these,

2:15

unlike other cysts and pancreas, tend to be true epithelial cysts.

2:19

So the epithelial-lined (true) cyst

2:21

to the pancreas. You also see serous cystadenomas.

2:24

We know what that looks like now, and it's very tough to know if some

2:28

of these are true cysts, or some of this could represent serous cystadenoma.

2:32

But luckily, true cysts and serous cystadenomas are benign,

2:35

so we don't worry about them.

2:36

The one thing you can see in these patients

2:38

are neuroendocrine tumors as well.

2:39

And so you need to be on the lookout,

2:41

particularly in your post-contrast sequences,

2:43

to make sure you're not dealing with any neuroendocrine tumors.

2:45

Because, of course, those are something that we need to worry about.

2:49

But besides that, you see disease in other organs,

2:52

and the kidneys here, he/she...

2:53

This patient had partial nephrectomy,

2:54

but you can see there's multiple renal neoplasms,

2:56

which is characteristic of vulnerable

2:58

and multiple bilateral renal neoplasms, that is.

3:01

You can see a pheochromocytoma, which is another finding

3:05

you can see in patients with Von Hippel-Lindau.

3:08

In the epididymis, in male patients, you can see cysts or papillary cystadenomas.

3:12

In the head and neck region, you can see endolymphatic sac tumors.

3:15

And in the CNS, hemangioblastomas.

3:18

They can happen in the cerebellum brainstem.

3:20

But one area that I always look for is in the spinal cord.

3:23

So you may end up getting cases where you're reading the abdomen,

3:26

look at the spinal cord and you may just see a little focus of enhancement.

3:31

If you do see that, that's probably going to be a hemangioblastoma.

3:35

Now, a lot of these patients end up

3:36

getting dedicated thoracic spine, lumbar spine MRI,

3:39

so you don't necessarily need to make that diagnosis.

3:41

But if you have endonovo case, it's good to know where,

3:45

look at the kidneys, adrenal glands, pancreas.

3:47

But if you really want to take it to that next level,

3:50

sort of completing your understanding of the disease and adding value,

3:54

look at the spinal cord to see if you see any tiny hemangioblastomas as well.

3:58

They can also get choroid plexus papillomas

4:01

in the CNS, under the CNS category.

4:04

So this is a patient with Von Hippel-Lindau, who gets followed regularly with us

4:09

to make sure that things are under control, and that these neoplasms aren't growing.