Interactive Transcript
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This is a 14-year-old child
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with neurofibromatosis type 1.
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Seeing an axial T2-weighted image,
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we can see this ovoid hyperintense area.
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And centered in the region of
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the left globus pallidus,
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we can see two patchy areas of signal
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abnormality in the right globus pallidus.
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If we look in this post-contrast
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T1-weighted image, we see this
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subtle halo of hyperintense
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signal around these areas.
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But before we refer to these as
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being areas of enhancement,
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we have to look at the pre-contrast T1.
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There is a hyperintense rim on
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pre-contrast T1-weighted imaging.
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So, there is no enhancement associated
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with these areas.
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They just happen to be hyperintense.
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So, looking at all the sequences is important.
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Now, if we look at this axial image on T2,
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we can see the two mammillary bodies.
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There's the optic chiasm.
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Here's the left optic tract.
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Here's the right optic tract.
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Now, the right optic tract looks asymmetrically thicker.
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Now, subtle asymmetry can possibly be related
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to obliquity slice selection.
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But even independent of the asymmetry,
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this is too thick for the right optic tract.
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If we look at this in the coronal image,
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we look at the prechiasmatic segment
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of the optic nerves,
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and there's actually asymmetric thickening
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of the prechiasmatic segment of the left
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optic nerve going into the left
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aspect of the optic chiasm.
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Now, if we go posterior,
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we can actually see there's asymmetric
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thickening and enlargement
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of the right optic tract.
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The left optic tract at this location
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looks approximately normal.
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The right optic tract is thicker,
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it's indistinct,
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and we're not seeing it clearly.
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So, this is a right optic tract glioma in the
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setting of neurofibromatosis type one,
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and likely also involvement of the left
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aspect of the optic chiasm and also
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the prechiasmatic segment of the left optic nerve.
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Now, if we go anterior to the orbits,
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seeing a little bit of tortuosity,
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we're not seeing any discrete enlargement.
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Now in this image here,
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we're seeing asymmetric hyperintense signal
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in the right optic nerve in the midorbital
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segment without significant enlargement,
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without definite abnormal enhancement.
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Given the other multifocal areas,
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this area here has to be suspicious for
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additional involvement of
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an optic pathway glioma.
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So, this shows that the optic pathway gliomas
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in neurofibromatosis type 1can manifest
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in several different locations.
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The question then becomes if there is a
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normal appearing intervening segment
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of the optic nerve on imaging,
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is that segment uninvolved
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or just subclinically involved?
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And the answer is,
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we don't know. It could be either.
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It is possible that the different areas of
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involvement are multicentric involvement,
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I say multi-centric because it may arise in
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two different locations due to
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the genetic predisposition,
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or it could be longitudinal spread of
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the glioma along the optic pathway,
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but with more focal areas that are
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discontinuous of more fusiform enlargement
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and enhancement. It could be both.
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So, that's one reason why we just need to try
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and look at the entire optic pathway,
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describe it the best we can,
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and if there's any questions,
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review it with the clinical team,
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with the oncologists,
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or anyone else who may have questions about this.
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