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I'm back with my colleague,

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Dr. Ben Laser,

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talking about a 29-year-old with Von Hippel-Lindau.

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We've got an axial pre and post-contrast T1 FLAIR,

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enhancing nodule with a cystic mass,

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the most common type,

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35% of hemangioblastoma presentations.

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Although when the nodule has a cyst inside it,

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that's a little less frequent.

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And this one does.

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So, strictly speaking,

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it's a lower percentage, like 6% to 12% for this type.

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And then the solid type,

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which is the second most common,

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usually smaller.

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We've got one in the lateral cerebellar hemisphere,

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and we've also got another one down lower,

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so a second one.

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So now I would ask you,

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what do we do as radiologists now?

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What should we be doing besides giving a

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diagnosis or differential diagnosis?

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So the next step would be to actually image the

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entire neural axis, pre and post-contrast.

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Okay, let's do that.

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Let's pull down a cervical spine image from this patient,

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which was done with contrast.

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And we can scroll up and down with our eye,

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and scroll side to side with our mouse.

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And as we scan up and down with our eye,

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we come upon this thing.

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You might say to yourself, really?

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That small?

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Are you going to call a hemangioblastoma

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for a lesion that small?

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Well, the answer is yes, they are that small.

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Now, the major differential diagnosis here is maybe you're

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catching a small kink in some peel vessels,

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but they usually sit along the dorsal spine.

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They're usually like little cherry-red nodules

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right in the back of the spine.

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And a lot of surgeons will go after these

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because they bleed very readily.

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So if they bleed in the spine,

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it's a lot more catastrophic,

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where you don't have a lot of space than if they bleed

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in the cerebellum.

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That's not good either.

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But they are very vascular.

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So, often, they have to come out at a small size.

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So now we scan up and down, don't see any others.

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Let's check out the thoracic spine.

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Let's scroll back and forth.

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And there is a nodule right there.

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Very tough to see.

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I'm going to blow it up for you in a minute.

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There's a nodule that's a little more clear,

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also very tough to see.

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You're going to say,

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"Really? That small?"

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And the answer is,

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"Absolutely. That small."

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These have to be very carefully watched.

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There's one of the nodules right there.

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And let's zoom out and show you the second nodule.

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Here's the second nodule right here.

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It's a little more round, as opposed to, say,

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the peel vessels, which are more linear.

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Those are peel vessels there.

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Here is a nodule of Von Hippel-Lindau,

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another very small hemangioblastoma.

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So, there are different subtypes of hemangioblastoma.

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Type one is von Hippel-Lindau without Pheo.

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Patients have renal and pancreatic cysts,

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renal cell carcinoma.

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This is the most common type.

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The second most common type, or the type two, actually,

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is Von Hippel-Lindau with Pheo.

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So we break them down without Pheo and with Pheo.

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And you have 2A,

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islet cell tumors can occur here without cysts.

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2B, renal and pancreatic disease.

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So, in summary,

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what are the places that you look in Von Hippel-Lindau?

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You look in the spine.

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How about in the brain?

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What's the triad in the brain you got to look?

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Posterior fossa, cerebellar hemisphere,

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the eyes, retinal hemangioblastoma,

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and then the internal auditory canals

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and the temporal bone.

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Looking for?

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Endolymphatic sac tumor.

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All right, Pomeranz and Laser out.