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Multiple Hemangioblastomas, Von Hippel Lindau

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This was a gentleman who was complaining

0:02

about neck pain.

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And we did this study initially without

0:07

contrast. On the scans that you see,

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we noticed that there's relatively little in

0:13

the way of degenerative changes in the

0:15

cervical spine. On the axial scans, again,

0:19

not much was identified. However,

0:23

the patient had evidence of prior surgery.

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And so for that reason, we gave gadolinium.

0:30

On the post-gadolinium-enhanced scan,

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we saw areas of contrast enhancement on the

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posterior surface of the spinal cord in

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the intradural extramedullary space.

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So here we see one of the lesions at

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the cervicomedullary junction.

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Here we see another lesion on the

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surface of the spinal cord.

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And if I move the image up a little bit more,

0:55

down here, we also were worried

0:57

about some of the areas

1:00

of enhancement at the cervicothoracic

1:02

junction, as far as these enhancing nodules.

1:05

I'm just going to try to magnify that as well

1:08

and move this around so you could see

1:10

this a little bit better down here.

1:13

And this was our area of concern.

1:15

When we performed the axial scans,

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we again saw areas of concern that were within

1:22

the thecal sac but outside the spinal cord.

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So, intradural extramedullary.

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And we had one large nodule here,

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which was at that cervicomedullary junction.

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And then here,

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as we approached the thoracic region,

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we had small areas of enhancement, as well.

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So we started to go through a differential diagnosis.

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And when you see multiple nodules of

1:44

enhancement on the surface of the spinal cord,

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we were most worried about things like

1:48

subarachnoid seeding as the most common thing

1:51

that would occur here. And in addition,

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we were concerned about something that might

1:56

be infectious on the surface of the spinal cord.

1:59

However, fortunately, we did include portion of the

2:04

cerebellum in the scan.

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And as we go to the top of the scan,

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you'll notice that there is another lesion in the

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cerebellum that's faintly seen on

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the topmost scan. Of course,

2:15

the clinicians hadn't given us any history

2:17

about the previous surgery for this patient.

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And the only history we had was neck pain.

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This patient had had a previous

2:26

hemangioblastoma resected from the cerebellum,

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had an additional hemangioblastoma.

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And this was a patient with Von Hippel Lindau

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disease with multiple intradural extramedullary

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hemangioblastomas throughout the cervical

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and cervicothoracic junction.

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When we proceeded to scan a little bit

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more superiorly into the brain,

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we identified more clearly that

2:50

enhancing mass. And of course,

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there were additional ones.

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When you have a patient who has hemangioblastomas,

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I just want to remind you to look at two other areas

3:00

of the anatomy that are important in patients

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with hemangioblastomas and Von Hippel Lindau

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disease. That is the temporal bone,

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because you can get endolymphatic sac tumors

3:12

of the temporal bone in patients with

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Von Hippel Lindau disease. And additionally,

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you want to look at the retina,

3:19

because retinal hemangioblastomas or retinal

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angiomas can occur in association with

3:25

Von Hippel Lindau disease, as well.

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So any little focal area of enhancement,

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as you see here along the posterior

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margin of the globe,

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you want to consider the possibility of

3:36

a retinal angioma associated with Von Hippel Lindau.

3:40

Of course, in the abdomen,

3:42

you would be worried about the renal cell

3:45

carcinomas and pancreatic lesions.

3:47

So an example of multiple intradural

3:50

extramedullary hemangioblastomas in a patient

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with Von Hippel Lindau disease and

3:56

cerebellar hemangioblastomas.

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

Brain

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