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Dr. Schupack,

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this is a 14-year-old with diabetes insipidus,

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vomiting, and headaches.

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We've got many sequences here to share with you,

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but we're going to focus on the Sagittal T1.

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The sagittal T2 fast spine echo.

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And then obviously,

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there's contrast enhancement of this abnormality in

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the suprasellar and intrasellar territory, which makes

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that snowman-type phenomenon. And bear in mind,

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this is a pediatric case.

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It is a male, it's a man.

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So what's our differential diagnosis here?

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Right, so the sellar is not enlarged,

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so this is primarily a suprasellar

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mass enhancing in a child.

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So there is a substantial differential which could

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include things like germinoma, sarcoid, eosinophilic

0:55

granuloma,

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things of that sort that affect the stalk and are

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in that differential of diabetes insipidus,

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which this child does present with now.

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I would say from the surgical standpoint,

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nobody's going to like that idea very much.

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Nobody wants to dig into anyone's hypothalamus.

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So we're going to really go to lengths to try

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to make a diagnosis, including CSF studies,

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and just really correlating all the other things that

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we can in terms of age and whether there are other

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findings elsewhere in the brain that would help us.

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So I think that's kind of our differential.

1:29

Lymphoma, maybe, but given the age,

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I would say Germinoma is going to be up there.

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And I think the person that read it favored Germinoma

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and Eosinophilic granuloma. Had it been an adult,

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you might include things like neurosarcoid,

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although that can occur in any age range.

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Metastasis to the suprasellar region,

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especially breast and lung, by far, are big.

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And then when you have stalk involvement,

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there are some really weird things,

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like pituocytoma and not so weird things.

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Lymphoid hypophysitis,

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but that's more of an inflammatory infiltrative

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immune-mediated process.

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So this is a mass as opposed to a non-mass

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like enlargement of the stalk region.

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And in my simplistic mind,

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I think what you were saying is big,

2:15

suprasellar lesion, young, otherwise healthy person.

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It's a juicy suprasellar,

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but it's not really a suprasellar mass.

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Right. Almost everything we're seeing is up here,

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and maybe it's infiltrating down below, maybe not,

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but it's not producing a heck of a lot of expansion.

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So germinoma is something to strongly consider here,

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and diabetes insipidus is particularly prevalent in

2:37

patients that have germinoma and

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stalk-related disease. Now,

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I guess hamartoma,

2:43

the enhancement kind of rules that out.

2:45

Yeah, hamartoma is out.

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You like those to be more in the

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region of the tuber scenario.

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The epicenter should be a little further back,

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closer to the mammillary body.

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I think the epicenter is over here.

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Hamartomas don't enhance.

2:58

They're usually associated with.

3:00

Elastic seizures or behavioral change,

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or sometimes they're associated with growth delay,

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occasionally with epilepsy. But diabetes insipidus,

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it can occur with a hamartoma.

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So that certainly doesn't rule it out,

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but it points a little bit more towards germinoma.

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I'll tell you what else points a lot more towards

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germinoma besides the enhancement hamartomas don't

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enhance is this little funny nubbin of growth

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purely headed towards the third ventricle.

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You don't see that with hamartoma.

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And we know that germinomas like to

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seed the cerebrospinal fluid,

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even though these are more common in males,

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in the male gender, in the pineal region,

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in the suprasellar region,

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some people think a little more common in females.

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Some textbooks say males equal to females,

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but there's no major gender predilection.

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90% of these present before age 20,

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and our patient is 14, so fits that criteria.

3:55

Diabetes insipidus, super common.

3:58

You can check that box off.

4:00

Pan-hypopituitarism because of infiltration of the pituitary,

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which is probably occurring here,

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is another thing to be on your guard for.

4:08

They can present rather acutely with decompensation

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and cortisol due to the lack of ACTH,

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or they can even present with hypothyroidism

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due to a decline in TSH production.

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These things are incredibly radiosensitive.

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They're often curable.

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I know this patient did not get surgery.

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The patient got radiotherapy and chemotherapy, and

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there's a 90% ten-year survival for these.

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Anything else clinical about?

4:36

What about HCG and alpha-fetoprotein in these?

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Right.

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That's part of this workup for suprasellar

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but particularly very similar to a pineal region

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workup, which would be looking at CSF characteristics,

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germ cell lesions and things in the pineal region.

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So it's very common, as you mentioned,

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that the presentation of the suprasellar

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of the pineal region. In males.

5:01

That's the characteristic one with paranoid syndrome.

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Okay. Up gaze, retraction nystagmus.

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You might not have heard about that one.

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Light near dissociation. Okay.

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So you can kind of reacquaint yourself with that one.

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If we're thinking about germinoma, this is kind

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of a relation to the pineal region ones that's

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a good clinical pearl. And of course,

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germinoma associated with HCG elevation more frequent

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in the CSF. Occasionally, it can spill into the blood.

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You get a blood elevation,

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and alpha-fetoprotein points more to embryonal cell

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or teratocarcinoma as opposed to germinoma.

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So this is a germinoma.

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It's proven surgically. It responded extremely well,

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as they usually do to radiotherapy and in this case,

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also chemotherapy.

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There wasn't a lot of seeding present,

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and I'm ready to move on to the next one.

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How about you?