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Summary of Intradural Intramedullary Neoplasms

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I'd just like to review with PowerPoint a few of the

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important points I've made in showing you cases of

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neoplasms in the intradural-intramedullary space.

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So as I mentioned,

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ependymomas overall are the most

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common spinal cord tumor.

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They generally are better defined than

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astrocytomas. In the filum region,

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we think of them not as intramedullary

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but as extramedullary in location.

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They have a higher rate of hemorrhage, and may show

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that hemosiderin cap sign at the superior or inferior

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border of the mass. These tumors are associated with

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Neurofibromatosis type 2.

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Here is an example of a grade two ependymoma in which

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you have a mass which has multiple characteristics

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of cystic components, as well as

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solid enhancing components,

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and it generally, has a well-defined border

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at the C2 level with edema

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showing above that border.

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So really the edge of the tumor is at the C2

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level. This area above does not represent neoplasm

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but just cord edema.

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And this lesion has both a cystic component

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identified as non-enhancing, as well as the solid

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enhancing component and goes

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out of the field of view.

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Here is another patient who has Neurofibromatosis

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type 2. So we know it's going to be occurring.

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There are bilateral vestibular schwannomas

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with multiple meningiomas.

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And as we look at the spinal cord,

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we see a mass that has both a cystic, as well as a

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solid component, as well as multiple

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nodules of contrast enhancement.

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So this is Neurofibromatosis type 2.

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Here we have meningioma

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which I will be talking about

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in my Intradural

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Extramedullary Tumor Talk. Here's the cystic

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component with a solid component to this ependymoma.

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But there are multiple ependymomas in this case who

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has NF2. Again also demonstrated on

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the T2-weighted scan, both the cyst,

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the solid cystic component, and then more

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diffuse portion, as well as a meningioma.

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MISME, multiple intracranial schwannomas,

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meningiomas, and ependymomas.

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Astrocytoma is the second most common tumor in adults,

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the most common tumor in children.

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It's usually less-defined.

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It may also have cysts associated with it.

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It favors the cervical region as opposed to the

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lumbosacral region for the conus and filum

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ependymoma, and it is associated

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with Neurofibromatosis type 1.

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Here we have a patient who has a diffuse

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process which may actually be holocord.

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When you see a holocord,

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all of the cord involved lesion.

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We're going to suggest it's an astrocytoma.

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If we identify necrotic areas within the tumor,

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we may suggest it's of a higher grade and, therefore,

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more likely to represent a glioblastoma.

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You notice that this patient,

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by virtue of the signal

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intensity and picture of the vertebral bodies,

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is a child. And in children,

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astrocytomas predominate.

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Here is another astrocytoma in a young adult,

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again looks like the bone marrow signal of the

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cervical spine represents a younger patient

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rather than showing degenerative changes.

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So, an astrocytoma again,

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in a younger patient with both a solid, as well as

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cystic component but extending over multiple

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levels. This patient does not show

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cap sign of hemosiderin

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which would have suggested instead an ependymoma.

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Here is a grade three astrocytoma,

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so anaplastic astrocytoma.

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What would suggest a higher-grade tumor such as

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anaplastic astrocytoma or glioblastoma? For this,

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again,

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we need to look at the contrast-enhanced component.

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So if we look at the contrast-enhanced component,

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we see areas that look like they're not just cystic,

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but more angry-looking necrosis.

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So the sine qua non of the higher-grade astrocytomas

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of the spinal cord will be the necrosis and the

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irregular contrast enhancement that you may also

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identify on the T2-weighted scan with the area of

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necrosis being brighter in signal intensity

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than the rest of the tumor.

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Grade three astrocytoma in an adult patient.

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Numerous investigators

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have utilized diffusion tensor imaging and attempted

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to distinguish ependymoma from astrocytomas by

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virtue of their features on DTI and the

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diffusion tractography. This, again,

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is not quite ready for primetime.

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Most people would say that if the tracts of the

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white matter are displaced but not infiltrated,

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it's more likely to represent an ependymoma.

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But we can have higher-grade ependymomas that disrupt

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the fibers. In this article by Asim Choudhri,

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it suggests that the DTI,

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when it's showing the fibers displaced

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by the ependymoma or astrocytoma,

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you have a better prognosis than if they

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are infiltrated. So on the left,

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we have a case where the fibers are displaced

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outwardly by the tumor,

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whereas on the right-hand side, you

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have infiltration of the fibers.

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So this is more of an infiltrative

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pattern of the fibers.

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Whereas here, you see that the fibers are displaced

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outwardly and the tumor resides in the center of the

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spinal cord. Let's move on to hemangioblastoma.

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Hemangioblastomas, as I mentioned, may be in the

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spinal cord or they may be pial-based.

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When they're in the spinal cord,

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they're usually along the periphery where you

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see a mural nodule associated with cysts.

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If you have flow voids associated with

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a tumor in the spinal cord,

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we're going to suggest that it

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represents hemangioblastoma.

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The hemangioblastoma intensely enhances to a greater

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extent than the ependymoma and astrocytomas, and

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by and large, they may be posteriorly located.

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We.

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Remember as well that these may be associated with

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multiplicity indicative of Von Hippel-Lindau disease.

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Spinal hemangioblastomas favor the thoracic region,

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astrocytomas,

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the cervical region, ependymomas, the filum

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terminale, and lumbosacral region.

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I gave you the rule of one-thirds.

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One-third of patients with spinal hemangioblastoma

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have Von Hippel-Lindau disease,

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and about one-third of patients with Von Hippel-Lindau

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disease will have a spinal hemangioblastoma.

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This means that two-thirds of spinal

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hemangioblastomas occur de novo without

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Von Hippel-Lindau disease.

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So it's more common without Von Hippel-Lindau

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disease than with Von Hippel-Lindau disease.

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Here is our classic example of a hemangioblastoma, a

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well-defined lesion on T2-weighted scan that shows

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a mural nodule which is showing contrast enhancement.

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Let me just highlight that on the post-gad T1-

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weighted scan. Here is that mural nodule

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which was not evident as much on the pre-gad,

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but you might have suggested it

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on the T2-weighted scan.

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Another example of hemangioblastoma

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with cyst and nodule.

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This is the case that I showed

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as part of the case review.

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And this may or may not be associated

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with a mural nodule

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cystic lesions in the posterior fossa representing

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cerebellar hemangioblastoma

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in a patient with Von Hippel-Lindau disease.

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I showed a separate case of a lesion in the conus

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medullaris that had cyst and solid

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nodular enhancement.

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This one I like a little bit better because

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as you can see, in this case, Dr.

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Yusum does not lie. Cyst

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and nodule.

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So this is the cystic component, and

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this is the nodule component.

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And remember, I said that with Von Hippel-Lindau

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disease or hemangioblastoma,

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you can have both intradural intramedullary lesions,

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as well as intradural extramedullary lesions.

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And here is that enhancing lesion in the cauda

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equina nerve root, so below the

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level of the spinal cord.

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This would represent intradural extramedullary pial-

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based nerve root hemangioblastoma associated with

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Von Hippel-Lindau disease by virtue of

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the multiplicity of the nodules.

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Here's some information about

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Von Hippel-Lindau disease.

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Remember that you can get retinal angiomas,

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as well as abdominal imaging findings

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which include renal cysts,

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renal cell carcinoma, pancreatic cysts,

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pancreatic neuroendocrine tumors,

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pheochromocytomas, and endolymphatic sac tumors.

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I did not show an example of metastasis

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as one of the cases.

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This is probably the fourth most common tumor of

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the spinal cord after Von Hippel-Lindau disease.

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Of the lesions that cause mets to the spinal cord,

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lung cancer is most common,

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breast cancer is the next most common,

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and they usually occur in any part of the

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spinal cord, be it cervical, thoracic, or lumbar.

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This is an example of two different patients

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who had metastases to the spinal cord.

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In general,

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they show solid enhancement and they may be

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difficult to distinguish from primary cord tumors.

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So this is three different patients.

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One showing a solid met from lung cancer,

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another showing a met from

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breast cancer.

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And this is the axial scan before

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and after radiation therapy

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showing the treatment effect on the metastasis.

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This was the lipoma that was demonstrated being

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a lesion that kind of spans the tumor versus

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a congenital lesion within the spinal cord, and

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demonstrating it with CT scan and fat-sat post-gad.

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So on the fat-sat post-gad T1-weighted scan to the right,

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you notice nothing enhancing

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which is typical of a lipoma.

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Were we to see any enhancement,

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we might worry about something like a liposarcoma.

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

CT

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