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Cervical Spine Plexiform Neurofibroma in a Patient with NF1

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This was a child that was being evaluated for visual

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field deficits and visual blurring.

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On the MRI of the brain,

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we identified that there was a mass at the foramen

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magnum that was located in an

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intradural extramedullary location. Let's look at this.

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Here we have the mass.

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We can see that it is extramedullary at the

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cervicomedullary junction at the skull base.

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And we see the so-called meniscus sign with

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enlargement of the CSF at the border with the tumor,

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which identifies it as intradural extramedullary.

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On the axial T2-weighted scan,

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we again see the masses separate from the spinal

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cord. So this is our intramedullary compartment,

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but it is within the dural

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thecal sac, so it's intradural extramedullary

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in location, identified here as the bright lesion.

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On our post-gadolinium-enhanced pulse sequences,

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we can see that the abnormality shows contrast

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enhancement. When we look further inferiorly,

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we see that this lesion extends outside the spinal

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canal. So if this is the edge of our dural thecal sac,

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we see that this lesion is extending outside and

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actually is going into the pre vertebral space from

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the neuroforamen at the C1-C2 level.

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I'd like to point out that this lesion has central

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lower signal intensity and a periphery

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of brighter signal intensity.

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And this is our so called target sign.

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If we were to do a sagittal through this,

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we would see as brighter on the periphery and darker

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in the center, the so-called target

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sign of neurofibromatosis, or

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neurofibroma as opposed to a schwannoma.

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And in point of fact,

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this patient has neurofibromatosis type 1,

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and this is what we would call

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a plexiform neurofibroma,

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which is one of the characteristic features

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of neurofibromatosis type 1.

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This patient also had additional lesions in the

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cervical spine. And we're going to move,

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move from the current study to one that's three

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years later after the patient has had resection of

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that cervicomedullary mass. So on this example,

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we can see that on a sagittal and coronal scan,

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that this patient has additional

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lesions in the brachial plexus.

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So this is clearly outside the spinal canal,

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which is here,

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but this is a mass that's associated with

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the brachial plexus on the right side.

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As you notice,

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it has somewhat of a lower signal intensity in the

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center and a periphery of brighter area on the

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T2-weighted scan, so-called target sign

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once again of neurofibroma.

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You'll notice that there's an additional

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one further superiorly here.

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So this patient has neurofibromatosis type 1.

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Let's look at the lumbar spine quickly

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for any additional neurofibromas,

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and we don't see anything of concern here on the

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coronal initial scan and then on the axial scans,

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no additional lesions.

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So it's important to inquire and to investigate the

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entire central nervous system when we're dealing

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with a patient with neurofibromatosis type 1.

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So let's go back to the original question.

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This was a child who had visual field

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problems and visual blurring.

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Remember that neurofibromatosis

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type 1 is associated with

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optic pathway gliomas.

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So if we look back at the patient's optic

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nerves on the scans through the orbits,

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we would be examining it closely for any evidence

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of optic nerve glioma. In this case,

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the patient did not have it.

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So for those of you who are trying to remember this,

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what are the seven major criteria

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of neurofibromatosis type 1?

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Again, we have to do this from memory.

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We have cafe au lait spots. That's one.

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Number two,

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axillary freckling.

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Number three,

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optic pathway glioma.

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Number four,

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a spinal dysplasia,

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sometimes with sphenoid wing dysplasia.

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We have number five,

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family member with neurofibromatosis type 1.

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Number six, plexiform neurofibroma.

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And number seven, lisch nodules,

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which are identified in the globes.

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So this was indeed a case of neurofibromatosis type 1

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with multiple neurofibromas in the intradural

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extramedullary, as well as extradural space,

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as well as in the brachial plexus.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Spine

Pediatrics

Neuroradiology

Neoplastic

Musculoskeletal (MSK)

MRI

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