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Ependymoma Associated with NF2

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So because I've been lying to you, I decided to

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give you a little bonus case just to start

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out the neoplastic category of disease.

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Here we have a T2-weighted scan in the

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center and a sagittal STIR image to the right.

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As we scroll through the T2-weighted scan, you

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see that there is cord expansion beginning at

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the upper cervical cord at the C1-C2 level

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with areas of bright signal intensity

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scattered within the spinal cord.

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Those areas of bright signal intensity are

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better seen on the T2-weighted scan, and we

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see that the cord appears to be most

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expanded at the C2 level.

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If we pull down our T2-weighted scan,

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we again see the abnormal signal intensity within

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the spinal cord, and in the upper cervical region,

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it does look like the cord is somewhat expanded.

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So in this situation with the heterogeneous

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signal intensity within the spinal cord,

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we might suggest that this represents a

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demyelinating disorder because it does look

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like there are scattered areas within the spinal cord.

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However, we're in the neoplastic category.

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Fortunately,

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we have the post-gadolinium enhanced scan.

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As we scroll the post-gadolinium enhanced scan,

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we are delighted to find that the patient has,

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in addition,

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a mass in the posterior fossa which appears

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to be within the cerebellopontine angle cistern.

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And we look on the tentorium when we see

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abnormal enhancement of the tentorium,

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identifying that there is a meningioma of the tentorium.

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So when we have a cerebellar pontine angle cistern mass

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and a mass along the tentorium

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which appears to be a meningioma,

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and we have a mass which is infiltrating the spinal cord,

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we may come to the correct diagnosis

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of Neurofibromatosis type 2.

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Neurofibromatosis type 2 is associated

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with ependymomas of the spinal cord.

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So indeed, this patient does not have enhancing

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demyelinating plaques but instead has a

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diffuse ependymoma within the spinal cord,

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actually potentially multifocal.

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And you notice also that there is an

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additional schwannoma which is associated with

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the skull base here on the right side on the axial scan.

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These lesions are also well-seen on

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our post-gadolinium enhanced scan,

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in which case we do see an additional

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schwannoma within the neural foramen on the

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right side at the C2 level, as well as the

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areas of enhancement within the spinal cord,

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representing the ependymoma.

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So ependymomas typically are seen as

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more well-defined lesions with the exception being ,

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in those cases, of patients who

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have neurofibromatosis type 2,

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where the ependymoma may be multifocal.

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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