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Spinal Cavernoma

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This is a 25-year-old man,

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neck pain and stiffness,

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numbness in both hands,

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and a motor vehicle accident in December of 2014.

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This exam was done about five and a half months later.

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Just to get you oriented,

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we're going to scroll through some sagittal images using a

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sagittal series of water-weighted images.

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We've got a PD spur on the left,

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a T2 spin echo in the middle, and a T1 spin echo,

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non-contrast on the right.

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And I'm sure you all have locked

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into this area of puffiness,

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gentle mass effect that has occurred in which space?

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The intramedullary space.

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That should be one of the first things you do.

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You should decide whether you are dealing with

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something that is extradural narrowing

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the subarachnoid space.

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Something that would be intradural right here.

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That would expand the subarachnoid space above

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and below, or something in the cord itself.

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And if the cord is expanded,

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that would encroach on the thecal space.

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So this red area would get smaller on both sides.

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That's just basic blocking and

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tackling from myelography.

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And everybody needs to know that if they are going

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to be professional imagers at the expert level.

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So now we have to decide what to do with this thing.

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It's got a little bit of mass effect.

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It's in the upper cervical spine at about C2.

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What's the differential diagnosis?

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So in a case like this,

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any intramedullary lesion,

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first thing is going to be tumor.

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Is this a tumor? You need to rule out a tumor.

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Then you can think of all the other rarer types of

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things such as whether it's a vascular malformation

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or a demyelinating lesion. Okay,

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let's start with that.

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Let's start with those two

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demyelinating lesions.

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They typically don't have much mass effect.

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No. And the classic one is MS.

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And where do you see that?

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Usually in the cord,

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typically peripheral wedge-shaped type appearance.

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Front or back? Usually back.

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Usually back. Back or side.

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I think we both agree this thing's

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a little bit in the front.

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Now,

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one caveat is that MS is a sort of a progressive

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but yet stuttering, waxing and waning.

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There's remissions associated with MS.

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And unfortunately this entity which we're going to

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describe has that same type of clinical course.

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So that can be a little bit confusing.

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So at least clinically it's in the differential

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diagnosis. But radiographically? No, it's not.

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In fact,

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I think our thing looks a little

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nodular, almost like popcorny.

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Let me blow up the middle one.

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Just to sort of emphasize that.

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Just to go back to your point about MS,

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typically you would expect to see other lesions.

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So here we can see the pons.

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The majority of the pons, the medulla,

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and the rest of the cervical spinal cord,

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which there are no other signal abnormalities.

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Great point. Yeah. No skip lesions.

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So now I turn my next question to you.

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You're a young, hard charging,

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brilliant radiologist tumor is what everybody wants to

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know. The clinician wants to know, is this a tumor?

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Is my patient going to die?

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I think the patient wants to know that, too.

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So is it a tumor?

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And if it's not a tumor, why not?

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So how do you figure that out?

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So, one that Dr.

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Palmer has just alluded to is that

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popcorn-type appearance.

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So you have different heterogeneous T1 and

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T2 weighted signals in this lesion.

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The other things to think about would be in this case,

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there's not much mass effect.

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A, there's no cord edema at this point.

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B,

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the other things to look at would be,

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does it have a hemosiderin ring,

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or does it have hemosiderin staining?

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Let's check that out while you're talking about it.

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I've got a gradient echo here.

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It's not the prettiest, but there is some sclerosis.

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I'll blow it up right here.

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I think I may have a better one right there.

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And then on this

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gradient echo sequence, no worries,

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you get this Blooming artifact.

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Sure.

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So these are all signs that would point toward

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something that is not as aggressive as an intramedullary

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neoplasm. Yeah. And just for our audience,

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blooming refers to exaggeration of signal

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hypointensity when you go from a spin echo to

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a gradient echo, so the signal goes down.

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Another characteristic of Blooming is you get

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geometric distortion if you have iron deposition,

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paramagnetic phenomena when you go from

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a spin echo to a gradient echo.

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So the tissue positions will also start to shift,

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and you might even see something like a dark hole with

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kind of a lighter rim around the outside as part

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of a Blooming phenomenon. Here, though,

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there's no question we've got a dark siderotic area.

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It's dark, but it's not lossless.

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Lossless means there's absolutely flat out no signal.

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That's lossless, that's air. So low signal,

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but not lossless.

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So let's go back now to a summary of your thoughts,

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which are excellent.

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The degree of mass effect for the lesion size not

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great. Not a lot. There's really no edema.

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The lesion has this sort of popcorny look to it.

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The zone of transition is pretty tight.

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The signal intensity,

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when you look at the T2 is a little bit

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heterogeneous. It's got hemosiderin staining,

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and it doesn't have a lot of length.

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When you think about something like ependymoma,

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which would come to mind anytime something

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has siderotic staining, it's got length.

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When you think about an astrocytoma,

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it looks like a cigar, you know,

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in the spinal cord,

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it also has length. So.

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The shape isn't good for that.

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And an intramedullary metastasis,

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which is most common from lung and breast,

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tremendous amounts of edema and swelling.

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I've seen many of them.

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They usually occur in the thoracic and lumbar area.

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So we're into something different and in this case,

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we're into a low flow state, vascular abnormality,

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namely a cavernoma.

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So let's talk a little bit about cavernomas.

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They're three to 5% of all cavernomas are in

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the spine. Most of them are in the brain.

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But the brain ones have a very different natural

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history. They usually don't bleed.

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And it is said that they can be acquired.

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You can get them from trauma,

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you can get them from radiation.

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So they can develop throughout life.

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The ones in the cord, there's more controversy about.

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Some people think they can be acquired,

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but there's a whole nother subset of scientists that

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believe that these are familial or genetically

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mediated. And in fact, when they are multiple,

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20% will be familial.

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And this has a much higher incidence in

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people of Hispanic extraction. Now,

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another difference between the brain ones,

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the brain ones don't bleed.

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These bleed we already alluded to the fact

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you get this sort of stuttering,

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waxing and waning phenomenon clinically, like MS.

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And then another very different characteristic

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is these don't calcify.

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The ones in the brain almost always calcify.

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So that's kind of strange.

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And then cavernomas tend to occur more in women.

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And this happens to be a man.

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And bizarrely enough,

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we showed some dural AVFs and those were women.

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So we showed you the opposite in terms of sex,

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of what you would expect.

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There's normal dural AVFs, men and cavernomas women.

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Just so happens that strange things happen.

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This is a cavernoma and it's occurred in a man.

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So in terms of bleeding, we said these things bleed.

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It's kind of a waxing and waning stuttering bleed.

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They bleed about one to 5% per year and they like the

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thoracic spine best, about 50% in the thoracic spine,

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40% cervical, and very uncommon in the CONUS,

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about 10%. So a cone lesion whole differential, right?

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Yes. When you think CONUS,

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what do you think about myxopapillary ependymoma

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would be the first thing that pops the most common

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tumor that could happen in the spinal cord?

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Sure.

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Or you could even relate it back to previous

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discussions of a vascular malformation,

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depending on the characteristics. Agreed.

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And myxopapillary ependymomas,

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they have myxoid tissue inside, but they also bleed.

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So that's a little bit confusing because the

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myxoid tissue is sort of high on T1 weighted images.

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The bleed is high on T1.

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So that can get a bit confusing but a lot more.

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Mass effect. Unfortunately,

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this lesion is pretty uncommon in the CONUS.

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I don't have any other comments about this.

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These do get resected sometimes when they bleed.

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In fact, they have to be resected.

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I will say there are a couple of other

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lesions that I didn't mention.

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One specifically that I think is important,

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and that is the hemangioblastoma.

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These are usually well and they're like little tiny

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nodules. I made a gray little spot there.

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I don't think that's good enough.

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I need something a little brighter

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to please the audience here.

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So you see these little nodules kind of studding

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the back. And when a surgeon looks in there,

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that's exactly what they see.

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They see a cherry red nodule right along the dorsal

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peel surface. And there's usually more than one.

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So even though these are both vascular lesions,

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they look very different from one another.

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Hemangioblastomas are multiple.

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They're associated with Von Hippel-Lindau syndrome.

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Cavernomas are not.

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I don't have any other comments in this case, do you?

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So the only thing I would comment on is going back

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to the history of a motor vehicle accident.

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The one thing I would probably not do is to

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confuse this with spinal cord trauma.

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What would spinal cord trauma look like?

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It would essentially you would have

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especially remote six months,

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five and a half months ago you would see a focal

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myelomalacia type signal within the spinal cord

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which is contracted or atrophied spinal cord.

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In this case,

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the lesion is actually it's puffed

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out the spinal cord.

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So to attribute this to a traumatic event

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would be misleading or wrong diagnosis.

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I think that's a great point.

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And I think a general radiologist reading this in a

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stack of films with that history

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would go down that pathway.

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So the fact that there is mass effect is really

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important. I think you've articulated it very well.

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Let's move on to another case, shall we?

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Vascular

Spine

Neuroradiology

Neuro

Musculoskeletal (MSK)

MSK

MRI

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