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Hemangioblastoma Part 1

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

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nausea, vomiting, and headache.

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I'm Dr. Stephen Pomeranz.

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This is a young stud, neuroradiologist, Dr. Ben Laser.

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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 T1 3D.

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So T1 appearing, I believe it's a T1 FLAIR.

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There's an axial view of a cystic mass with a nodule

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associated with it. A T2 weighted image.

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There's our nodule 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 T1 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 the first thing that would pop into my

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mind would be Hyang Blastoma um cyst

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with a mural nodule. Uh If the,

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if the patient was younger than 2015 or so,

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then Pilocytic astrocytoma would be on the differential. Yeah,

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

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almost never get hemangioblastoma.

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Even Von Hippel-Lindau cases,

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they usually show up a little bit later than pilocytic

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under age 10 strongly favors pilocytic under age five or six.

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I mean,

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overwhelmingly the diagnosis is going to be pilocytic.

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So human AOB blastoma in that age group, very rare.

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Conversely,

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it's not very rare to have a pilocytic in a 30-year-old.

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But certainly hemangioblastoma rises up to the top.

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It is the most common neoplasm in the

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young adult of the posterior fossa.

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And 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 pilocytic or

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hemangioblastoma? In this case, hemangioblastoma?

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Actually on the T1 weighted sequence, you can,

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one thing you want to look at is the appearance

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of the cystic fluid component. In this case,

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comparing it to the fourth ventricle,

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you can see that the cystic component of the lesion is

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slightly hyperintense compared to the CSF. Yeah,

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

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Some of the things that I might use

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to help rule out pilocytic tumor.

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One of the things is the nodule if the nodule has a

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cyst inside it and this one does that speaks more to

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hemangioblastoma and if the nodule has voids little

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punctate flow voids inside, which you can sometimes see,

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I don't see it here but you see these little

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dark flow voids and they would be black,

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then pilocytic is virtually out as a potential diagnosis.

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The other thing you can do is make sure that you don't

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have any other lesions because usually you don't have

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multiple pilocytic astrocytoma. But usually in VHL,

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you do have many or more than one hemangioblastoma.

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And even if you don't have on hippo linda,

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chances are you might have it.

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So if you find the hemangioblastoma isolated without

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a diagnosis of VHL, you better be looking for VHL.

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So let's scroll and see if we can

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come up with any other lesions.

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It looks like somebody was fishing around here in the

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left cerebellum. So that's kind of suspicious.

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Let's go to the contrast enhanced image and boom.

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All of a sudden we find one in the lateral cerebellar

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hemisphere. What do you think that is?

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It looks like a solid solid hemangioblastoma?

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So we had one with a cyst with a cyst inside the cyst,

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a cyst in 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 seen about 35% of the time.

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This type, about 33% of the time. Let's see.

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Can we find any others?

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Let's scroll a little bit because

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very often when they're small,

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they'll show up as like a little cherry red nodule

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visually and our M they're very vascular and there's our

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cherry red nodule. Now, as part of this vignette,

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we'll come back to this case in a

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minute in a separate vignette.

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Let's draw some of the appearances of Von Hippel Linda.

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One is just a simple pro tenacious cyst

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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

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kind of nodule associated with it.

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Then you have a cyst with a mural nodule

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and a cyst within that mural nodule.

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And we kind of have that here. So if you are,

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if you're very O CD and very strict about it,

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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 and

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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

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a solid lesion that's about 12%. Now,

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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. Now,

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the solid mass in my experience is always much smaller

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than the cystic mass with a nodule. And in fact,

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that's not just an experience,

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I think you can take that as gold because if these

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things get this big, you know, if they get really big,

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then they're going to bleed.

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So the reason you don't see them like this

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is they 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.

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So that is the appearance of hemangioblastoma.

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Let's move on to the next vignette and discuss this

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case in a little more detail. Shall we?

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We shall.

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Laser and P out.

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Syndromes

Pediatrics

Neuroradiology

Neoplastic

MRI

Brain

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