0:00

Dr. Schupack, this is a four-year-old complex case.

0:03

Very large mass. It's a supersellar mass,

0:07

and it's a proven

0:11

adamantinomatous craniopharyngioma.

0:14

The other type is the Papillary type.

0:16

The adamantinomatous type is cystic, may have calcium in it.

0:19

The Papillary type more frequently seen in adults,

0:23

more solid-looking and does have solid-type enhancement.

0:27

This one barely enhances around the outside.

0:30

It's a T1-weighted image.

0:31

We've got a flare here,

0:32

which I know you're going to comment on.

0:33

The ventricles are a little big with

0:35

some periventricular high signal.

0:37

Then there's this funny-looking abnormality that's hanging

0:41

down from the right middle cerebral artery,

0:43

which we don't know what it is.

0:44

The case is absolutely proven.

0:46

And then when we go posteriorly along the back of the lesion,

0:49

we see that there's a developmental venous anomaly in the

0:52

right cerebellar hemisphere and the

0:54

right white matter or linea alba.

0:57

So let's talk about what to do with this and your approach to

1:01

it take over. Well, there's going to be a couple of phases.

1:04

Dr.

1:05

Pomerance has done the first phase that the radiologist

1:08

can help with, which is arriving at a diagnosis.

1:10

We know what that is.

1:13

But really,

1:14

where the radiologist can really be valuable is the next phase

1:17

of figuring out, hey, what has to happen now and how quickly.

1:21

All right,

1:22

so the first thing that your attention is going

1:24

to be drawn to is regardless of the mass,

1:27

you're going to get focused on that.

1:29

But, boy,

1:30

we got an immediate problem right here that's going to deserve

1:32

a phone call, which is obstructive hydrocephalus.

1:36

Now, you can see ventricular capping, all right?

1:39

Now, this is a young patient,

1:41

but you're always seeing vascular disease

1:43

signal around the ventricles.

1:45

But this capping pattern transapendymal spread of CSF.

1:50

The temporal horns are big.

1:52

So this person, before they need anything,

1:55

needs some control of their CSF obstruction,

1:58

whether it be an external ventricular drain or a more

2:02

definitive shunt, depending on what the ultimate plan is.

2:05

But the physician who's taking care of this patient needs to

2:09

know about that before we kind of get to anything else.

2:13

Yeah,

2:13

and you're never going to get capping of that extent in a

2:15

child. Never. Right. Their ventricles are going to be tiny,

2:18

right, in a little kid like this, right?

2:20

Sure. And so giant ventricles.

2:22

So that's issue number one in terms of urgency.

2:27

And what's going to happen before anything else does is making

2:30

sure that while we're all thinking about this thing,

2:32

that this poor child doesn't herniate.

2:35

Okay? Now, so we talked before about the lesion itself,

2:39

and just this is a really good example.

2:41

Huge lesion, right?

2:42

But different than another lesion that we had seen earlier,

2:45

because the cella is not big, okay?

2:48

And there's pituitary tissue.

2:50

You mean the sella turcica, the sella turcica.

2:53

The sella is not enlarged,

2:54

so there's no transphenoidal thing here because

2:56

there's no place to work through.

2:58

Okay.

2:59

And that's just not where

3:00

the action is.

3:00

If you could let me just take over for a minute so I can blow

3:03

it up a little bit so we can show the pituitary gland,

3:06

which is separate from it.

3:07

And the sella really isn't big for the lesion size.

3:10

There's the gland right there.

3:11

There's the gland right there.

3:13

So it is clearly separate from the pituitary gland.

3:15

Sorry, go ahead. Sure. So as far as surgical options.

3:21

So you can't go do anything from the transphenoid approach.

3:25

So it's going to be a transcranial approach of some sort.

3:28

And what are the goals going to be?

3:31

Well, you got a very important goal right here,

3:34

which is decompressing the brainstem.

3:37

Also, this person presented with visual loss,

3:40

so decompressing this portion of it,

3:43

and the chiasm and optic apparatus are in there somewhere.

3:46

This case is so bad that we can't even tell where.

3:49

But trying to get some decompression because this

3:51

in due course is going to kill this person.

3:53

Sure. And brainstem compression is how that might occur.

3:58

Yeah. And just you reminded me,

4:01

one of the signs of craniopharyngioma is the mustache sign,

4:04

where the optic apparatus is draped over the lesion

4:08

as a mustache, seen in the coronal projection.

4:10

But you sort of have a mustache-like phenomenon right here.

4:13

I can draw over it right there.

4:15

Looks a little bit like a downward-turning mustache.

4:18

So I'm sorry. Go ahead.

4:20

And so what is going to help with the thinking

4:23

about what the definitive treatment is?

4:25

So this has an effect on the basilar artery.

4:28

Okay. Now, we were thinking that there's facial weakness,

4:31

but it's a little hard to explain

4:33

because when you get down there,

4:35

we don't really have

4:39

7th and 8th nerve complexes.

4:41

So it may be some sort of brainstem phenomenon that we're

4:43

looking at, but at least we don't have to decompress that.

4:47

But there's retroclival extension. Okay.

4:50

The basilar artery. Meaning if you start taking this out,

4:52

the basilar artery is going to be

4:54

at the end of your dissection.

4:55

You certainly don't want to have a problem with that.

4:57

It's kind of weird because the facial you just drove by the

5:00

facial nerve nucleus right here is the facial colliculus

5:03

bump right in the floor of the fossa.

5:05

Okay. The facial colliculus right there.

5:09

There's nothing there.

5:10

So it's hard to explain why the child has a

5:14

facial nerve palsy, but go ahead.

5:15

Right.

5:16

Middle fossa is a problem.

5:18

And you said that there's a lesion out here.

5:20

I think I would investigate that a little bit.

5:23

Meaning because your approach is probably going to be

5:25

from this side and it's right next to the lesion.

5:29

It may be some unusual manifestation of the lesion itself,

5:32

some sort of daughter cyst.

5:34

I just want to be sure it's not vascular.

5:36

Sure. Okay.

5:36

Because we're going to need to protect this

5:38

middle cerebral complex on our way in.

5:41

And in thinking about it, I don't think it was vascular.

5:43

Now,

5:44

that I'm looking at it in its totality because it

5:46

does match the signal intensity of the lesion.

5:49

It matches it over here.

5:50

I think we briefly considered that it could be aneurysm draped

5:53

down from the M one segment or the M two segment of the

5:57

middle cerebral artery. But the patient had an.

6:00

Eventful resection,

6:01

and nobody reported any vascular lesions other than a

6:04

developmental venous anomaly in the back of a

6:06

cerebellum which was not operated on.

6:08

Okay.

6:09

As I say, in a case like this,

6:11

where there's very obvious abnormality,

6:13

most of my report is about describing the relation of

6:16

the lesion to things that you want to know about.

6:18

For example,

6:19

the anterior cerebral complex kind of riding on top of the

6:23

lesion because that may affect somebody's thinking

6:26

about how they're going to deal with it.

6:27

So very difficult problem.

6:30

What the definitive treatment is going to be is unclear,

6:33

but there's definitely an urgent component to it.

6:36

And then using the information,

6:39

thinking through how you can get there and what might be the

6:43

issues in terms of anatomy going to be really helpful

6:45

to clinicians. Great. One final parting question.

6:48

As a snookie radiologist,

6:50

not a clinician

6:53

with a lesion that's separate from the pituitary gland,

6:55

does that preclude you going from the bottom now

6:58

that you know it's a supercellular mass?

6:59

Well,

7:00

there are extended skull base approaches these days where they

7:04

endoscopic approaches where they actually mobilize

7:08

the pituitary and go through the clivus.

7:10

Wow. Okay, so there could be some things like that.

7:13

But the fact is, unless it's a primarily cellar lesion,

7:16

the cellar is going to be small.

7:18

So here's this giant lesion, tiny little cellar.

7:21

I mean, it's going to be a little bit hard.

7:22

So there could be some role for a skull based approach,

7:25

at least in part of this.

7:26

But classically speaking,

7:29

if you're going to do transphenoidal and

7:31

there's a supercellular component,

7:32

you need kind of a big cellar to work through.

7:35

And this one, say,

7:36

different than the last one that we discussed.

7:38

Right? So that one had giant cellar.

7:40

The thing is into this phenoid is right there in front of you.

7:44

This thing is giant thing with a little tiny hole.

7:46

Going to be tough. Got it.

7:48

All right, well,

7:48

let's move on to our summary vignette on craniopharyngioma,

7:51

shall we?