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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.