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Additional Spinal Canal Manifestations of VHL

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Well, I'm going to continue my theme of the mea culpa

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and admit that I've lied to you yet again.

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So let's look at this case and identify one of

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Dr. Yousem's fabrications.

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So here on this post gadolinium-enhanced scan

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of a patient who has known von Hippel-Lindau

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disease, where the patient has already had a

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hemangioblastoma resected, we see that there

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are contrast-enhancing nodules once again,

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bilaterally in the cerebellum, both at the

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vermis as well as in the lateral hemisphere.

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You note that there are

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post-operative changes from a previous

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hemangioblastoma that has been resected.

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So as I scroll through the brain

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tissue, when I'm looking at a patient

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who has a known hemangioblastoma,

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a couple of things to remind you.

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Remember that you can have retinal

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hemangioblastomas, so look at the back

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of the orbits and make sure there's

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no areas of contrast enhancement.

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And remember that the patient who has Von Hippel-Lindau

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disease also has a predilection for

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endolymphatic sac tumors, which was demonstrated

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on the previous example of a Von Hippel-Lindau

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patient who had a left temporal bone lesion.

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Let's move to the spine and try to

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identify a spinal cord hemangioblastoma.

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And I told you that these lesions

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are typically cystic and solid.

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So as we scroll through the spine,

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we don't see anything within the spinal cord.

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Here's the lie.

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So I'm going to magnify this case

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and demonstrate another manifestation

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of von Hippel-Lindau disease.

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And that is, you can get intradural

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extramedullary masses associated with

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Von Hippel-Lindau disease and hemangioblastomas

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on the surface of the spinal cord.

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And you may see these down in the

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cauda equina nerve roots as well.

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So when you have multiple hemangioblastomas

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of the spinal, of the, uh, von Hippel-Lindau

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disease, you have to look not only in the

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cord, but intradural extramedullary for the

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potential of little seeds of hemangioblastomas.

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Now I'm using the term seeds here, not to

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imply that these are subarachnoid seeding the

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intracranial hemangioblastoma, but instead, small

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PIL-based (PIAL) hemangioblastomas

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on the surface of the spinal cord and

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potentially in the cauda equina nerve roots.

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So, another manifestation of Von Hippel-Lindau

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disease and hemangioblastoma of the spinal cord.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Spine

Neuroradiology

Neoplastic

Musculoskeletal (MSK)

MRI

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