Interactive Transcript
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You may recall that I referred to spinal
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hemangioblastomas in the intradural
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intramedullary talk.
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However, spinal hemangioblastomas may also occur on
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the pial surface of the spinal cord,
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not within the spinal cord.
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And they also may occur below the termination
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of the conus medullaris in the
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cauda equina nerve roots.
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So we do see intradural extramedullary
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hemangioblastomas, as well as intradural
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intramedullary hemangioblastomas.
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Remember that spinal hemangioblastomas
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have the so-called one-third rule.
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That is, that one-third of patients who have
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Von Hippel Lindau disease,
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have spinal hemangioblastomas,
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and one third of patients with a spinal
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hemangioblastoma have Von Hippel Lindau disease.
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Von Hippel Lindau disease, you may recall,
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is associated with cerebellar
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hemangioblastomas, as well as hemangioblastomas
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of the spinal canal.
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And they are also associated with renal cell
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carcinoma and pancreatic tumors and cysts,
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et cetera.
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The characteristic feature of the spinal
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hemangioblastoma in the spinal cord is
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having a cyst and a nodule in 75%.
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Those that occur on the cauda equina nerve
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roots are almost entirely solid in their appearance.
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Here we have a patient where we have the
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T1-weighted scan to the left and the
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T1 post-gadolinium-enhanced scan to the right.
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We are at the lower thoracic region and
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what we see are actually two lesions.
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You can see that this lesion here is within
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the spinal cord and it has a cystic, as well as
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a solid component and the solid component
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is showing enhancement.
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So we are at the
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T12-T11 level where we have the spinal cord.
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So this is intradural,
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intramedullary hemangioblastoma.
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However, at the L2 level,
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we see an additional enhancing lesion.
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This, too, is a hemangioblastoma,
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but this would be characterized as intradural
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extramedullary. As I said, occasionally,
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you will see tumors that are
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just on the surface of the spinal cord,
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and those are also considered intradural
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extramedullary hemangioblastomas.
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So while the vast majority of intradural
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extramedullary hemangioblastomas occur below
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the termination of the spinal cord,
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you can occasionally get them on the surface
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of the spinal cord, generally posteriorly,
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and favoring the thoracic region.
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So this is an example of a patient
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who has Von Hippolindal disease, Hippel Lindau
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multiple hemangioblastomas,
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including one that's intradural intramedullary
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and then an additional one that is intradural
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extramedullary on the cauda equina nerve roots.
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