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So here's a tough case, Dr. Shupack.

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This is a 13-year-old young lady,

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who's got growth hormone deficiency

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and also has a delayed bone age.

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Her bone age is about 11 and she's 13 years of age.

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I've got before you a sagittal T2,

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fast spin echo, a sagittal T1 without contrast on board,

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and then contrast is on board on this sagittal T1.

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Let's scroll a little bit.

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And there is a defect in the pituitary gland, causing a little

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bit of upward convexity. The T2 is not all that impressive.

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I mean,

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there's some slight increased signal intensity corresponding

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to that locus. And then there's also on the T1,

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a little bit of maybe proteinaceous or slightly high

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signal intensity in the center of the gland.

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But it's not a typical classic cyst like you might see

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in a pituitary cyst or pars intermedia Rathke cyst.

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So it's a weirdo. And she's got this hormone lab near madly.

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So what do. Do with us.

1:01

Right. And you highlighted already,

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we had seen in the previous video a pituitary

1:08

cyst or pars intermedia cyst. Boy,

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that looks and this one looks a lot like that size.

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Very similar. Not too much different in location,

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but I would say with a couple of sort of important subtle

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but important differences. So, this one, first of all,

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the location is very similar to that last case,

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but it's a little more anterior in the gland.

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So, as opposed to being in the pars intermedia,

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where you expect a pars intermedia cyst,

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this 1 may be actually in the posterior

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portion of the adenohypophysis,

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which kind of makes you think along a different

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line that's a little too far forward.

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And you're referring to the pituitary cyst vignette.

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That's a companion to this.

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That's correct. Now, the other thing is,

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in previous

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expositions,

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we've talked about something called the pituitary tuft,

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which is a vascular structure in the center.

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So, if something is in the

2:00

Pars intermedia, it's going to push the tuft forward,

2:03

but on the coronal view, it should look pretty normal.

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But this is kind of in the center.

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It's sort of splaying the tuft.

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So that's another piece of evidence.

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You'd say, wow, this might not be in the pars intermedia,

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and it's also demonstrating a type of mass effect

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that's a little different than that last one.

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So we might start thinking along the lines

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of this being not that sort of cyst,

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but maybe lesion of the adenohypophysis itself.

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Now, another thing is,

2:29

you mentioned that we're talking about

2:31

an insufficiency syndrome,

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and if you measure the height here for a young female,

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it's probably a little bit on the skimpy side.

2:39

It is, yeah. I think I measured it out about 4447,

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and I know from our prior discussions you would expect it to

2:46

be about a millimeter or maybe even a little bit

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more than a millimeter taller than that,

2:50

which would go along with her possible

2:53

growth hormone deficiency.

2:55

Whereas this little lesion may be just an incidentaloma

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which we know. Occurs about 15% to 20% of the time.

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And you'd say, well, what if it's secreting growth hormone?

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Well, then you would have too much growth.

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Growth hormone not too little.

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So that doesn't make sense.

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This is probably going to be an incidental finding.

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And you were talking about the pituitary tuft,

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which gives me the opportunity to talk a little bit about

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the blood supply. And I'll use red for blood supply.

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The superior Hypophyseal artery kind of comes in from the

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carotid artery and then it sends some branches this

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way and comes down to the pituitary gland here.

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But mostly it's going to feed the neurohypophyseal axis in the

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back and it'll send some little branches this way as well.

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So you'll get a little bit of blood supply

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to the pars intermedia from it.

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But then you've got I'll use purple for the inferior

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Hypophyseal artery, which comes in this way,

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and it kind of bisects the pars distalis

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and the pars intermedia.

3:55

Give off some branches both ways and then it'll

3:57

loop up in this direction.

3:59

Give a little.

4:00

A supply to the to the median eminence

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and to the neurohypophysis as well.

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But it's considered more of a blood supply kind of to the

4:07

pars intermedia and the back of the pars distalis.

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But most of the pars distalis consists of the portal plexus

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which coalesces up here with these arteries as a venous system

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that kind of drops down this portal plexus of veins and feeds

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the pars distalis and the kind of enhance that way.

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In the back, you get earliest enhancement.

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Then a little bit of enhancement in the pars intermedia

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and then finally in the front in the pars distalis.

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So it's kind of a complex blood supply,

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but it's this portal plexus that creates the tuft.

4:40

So it's these descending vessels which

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I'll draw one more time in blue.

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They're coming down from the median eminence and

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from the hypothalamus. And here they are,

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and they give this little vascular blush.

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And as Dr. Schupeck has said in the intracellular region,

4:55

our thing is right in the middle of this blush now.

5:00

Prior vignettes.

5:01

We said that prolactinomas and growth hormone-secreting

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tumors like the lateral wing of the pituitary gland.

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They're more out here, right there,

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the ones that are kind of closer to the midline.

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Probably the most important one is the ACTH-secreting

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microadenoma. And this area is known as the mucoid wedge,

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where the ACTH-secreting adenoma may be located.

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So it's unlikely that this is an ACTH-secreting adenoma,

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but that would be something to consider in

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somebody that had more systemic symptoms.

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Any other comments regarding this case?

5:35

No. As I say, there's not compression,

5:40

there's not cavernous sinus invasion.

5:42

So really treatment would be based on endocrine

5:45

and whether there's a medical option.

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And I just have three other brief comments

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since it's a 13-year-old.

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If there was a proven adenoma in this case,

5:57

there is an entity called Familial isolated.

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Pituitary adenoma syndrome, also called FIPA syndrome.

6:03

It's autosomal dominant with lower variable penetrance.

6:06

I won't talk about the germline mutations.

6:10

The second one would be multiple endocrine neoplasia,

6:13

which everybody in medical school gets to learn because

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it's so interesting. MEN or men, type one.

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This is a mutation. In the menin,

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they get parathyroid and pancreatic tumors along with their

6:25

pituitary abnormalities. And then the carney complex,

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which is also kind of a fun one.

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It's a mutation in type one,

6:32

a subunit of protein kinase A 10% have pituitary adenomas,

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usually growth hormone-secreting ones.

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They get spotty skin pigmentation, myxomas,

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schwannomas pigmented, nodular,

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adrenal cortical disease causing Cushing syndrome

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in about 30%. So those are three familial.

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Early presentation pituitary adenoma syndromes.

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Let's move on, shall we?