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I'd like to share a case with you of a 29-year-old

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with nausea, vomiting, and headache.

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I'm Dr. Steven Pomerance.

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This is Young Stud Neuroradiologist, Dr. Ben Lasar.

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And we're talking about a weird case.

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A gentleman shows up age 29 with these symptoms

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and this MRI axial T one 3D.

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So T one appearing, I believe this is a T one flare.

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There's an axial view of a cystic mass

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with a nodule associated with it.

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A T two weighted image.

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There's our nodule

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and it might have a little cyst inside it.

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And then here is our cystic mass again

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after contrast on the T one flare and it's enhancing.

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So what would be some of your thoughts?

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What would be the differential here?

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So the first thing that I would take a

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look at would be the patient's age.

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So differential. If the patient was greater than 30,

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greater than 25, um, the first thing

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that would pop into my mind would be he angio Blas stoma.

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Mm-Hmm. Um, cyst with a neural nodule.

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Uh, if the, if the patient was younger than 2015

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or so, then pilocytic astrocytoma would

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be on the differential.

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Yeah. And then, you know, if you're under age 10,

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you almost never get, uh, he angio blasts, stoma,

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even von Hippo lindo cases,

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they usually show up a little bit later than pilocytics.

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So under age 10 strongly favors tic under age five or six.

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I mean, overwhelmingly the diagnosis is gonna be tic.

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So he angio blasts stoma in that age group, very rare.

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Conversely, it's not very rare

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to have a tic in a 30-year-old.

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But certainly Heman neoblastoma rises up to the top.

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It is the most common neoplasm in the young adult

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of the posterior fossa.

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Mm-Hmm. And uh, so you have to favor that diagnosis.

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And how about the appearance of it?

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Do you like the appearance for tic or heman neuroblastoma

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In this case? Uh,

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heman neuroblastoma actually on the T one weighted

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sequence, um, you can,

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one thing you wanna look at is the appearance

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of the cystic fluid component.

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In this case, comparing it to the fourth ventricle,

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you can see that the cystic component

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of the lesion is slightly hyperintense compared to the CSF.

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Yeah, it's a little grayer. It's a little grayer.

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Some of the things that I might use to,

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to help rule out pill acidic tumor, uh, one

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of the things is the nodule.

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If the nodule has a cyst inside it

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and this one does that speaks more to Heman neoblastoma.

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And if the nodule has flow voids, little punctate flow voids

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inside, which you can sometimes see, I don't see it here,

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but if you see these little do dark flow voids

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and they would be black, then pilocytic is virtually out

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as a potential diagnosis.

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The other thing you can do is make sure

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that you don't have any other lesions.

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'cause usually you don't have multiple pilocytic

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astrocytomas, but usually in Von Hippo you do have

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many or more than one.

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He angio blasts stoma.

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And even if you don't have on HIPAA endow,

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chances are you might have it.

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So if you find the he angio blasts stoma isolated without a

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diagnosis of VHL, you better be looking for VHL.

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So let's scroll and see if we can come up

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with any other lesions.

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It looks like somebody was fishing

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around here in the left cerebellum, so

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that's kind of suspicious.

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Let's go to the contrast enhanced image

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and boom, all of a sudden we find one in the lateral

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cerebellar hemisphere.

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What do you think that is?

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It, it looks like a solid, he angios

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A solid hest.

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So we had one with a cyst, with a cyst

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inside the cyst, a cyst and a nodule.

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We'll go through the appearance in a minute.

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We've got a solid looking one,

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which is the second most common type.

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This type usually, uh, seen about 35% of the time.

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This type about 33% of the time.

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Let's see, can we find any others?

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Let's scroll a little bit.

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'cause very often when they're small, they'll show up

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as like a little cherry red nodule, visually.

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And an MRI, they're very vascular.

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And there's our cherry red nodule.

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Now, as part of this vignette,

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we'll come back to this case in a minute.

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As separate vignette, let's draw some

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of the appearances of Von Hippo.

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One is just a simple pro tenacious

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cyst looking mass without a nodule.

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Another one is a cyst with a mural nodule.

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That's the most common type. That's about 35%.

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Then you get a cyst with some irregular kind

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of nodule associated with it.

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Then you have a cyst with a mural nodule

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and cyst within that mural nodule.

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And we kind of have that here.

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So if you were, if you're very OCD

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and very strict about it, this is actually this type which

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occurs about 6% of the time.

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Then you've got a solid lesion like this,

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and then you've got a cystic component within.

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So I'll make the cystic component green.

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So there's your cystic component inside a solid lesion

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that's about 12%.

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Now we'll go back to red here for a minute. Or orange red.

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And finally the last one,

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the second most common type is the solid mass.

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Now, the solid mass in my experience, is always much smaller

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than the cystic mass with a nodule.

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And in fact, that's not just an experience.

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I think you can take that as gold

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because if these things get this big, you know,

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if they get really big, then they're gonna bleed.

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So the reason they, you don't see them like this,

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is they've bled when they're about this size.

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So they've often been removed.

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They don't get to be this big. So tell me the criteria.

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Feed me the criteria for von Hippo Lindo.

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So the criteria for Von Hippo lindo

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includes CNS and retinal.

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He angio neuroblastomas, as we discussed, he angio,

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neuroblastoma, and one of the following, either renal cysts,

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pancreatic cysts, hepatic cysts, epidermal cysts,

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and even possibly lung cysts.

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Mm-Hmm. Uh, additionally, cy pheochromocytoma

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and renal cancer are additional criteria.

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Now there's another set of

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criteria related to family history.

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Mm-Hmm. What would be that

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Criteria? So the family history and

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one of the following,

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so hemangiomas, visceral lesions,

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pheochromocytoma and renal cancer.

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Alright. So in this, in this patient, you know,

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we're gonna focus on three areas.

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Uh, and that would be the, the globe, the,

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the ear, and the cerebellum.

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We've already started with the cerebellum.

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So what are we looking for in the ear?

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So in the ear you're looking for

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endo, lymphatic sac tumor.

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And what are we looking for in the globe?

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Either a retinal, a mass within the globe. Mm-Hmm.

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Uh, possible retinal detachment.

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Um, any enhancing, avidly enhancing lesion.

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Yeah. And so if somebody shows up

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with a retinal detachment,

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or say this is bulby, a shrunken globe,

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and they've got a cerebellar mass, like a cystic mass

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with a nodule, first thing you ought to think of is

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cerebellar retinal.

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He angio blasts, stoma, posis,

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otherwise known as von Hippo Lindo syndrome.

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Let's move on, shall we?

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Let's Azar NP out.