Interactive Transcript
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Dr. P here. This is a 34-year-old man who had a mass
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3 00:00:05,010 --> 00:00:08,119 on his foot that was biopsied, and the biopsy came
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back nodular fasciitis, and that is a good lesson.
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And the lesson is, when you have pathology in
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either the bone or soft tissues, you want a bone
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or soft tissue bone pathologist looking at your
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stuff because that was not the correct diagnosis.
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And he presents somewhat later with this mass.
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There's a few characteristics of
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this mass on T2, T1, and contrast
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enhanced T1 with extensive enhancement
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that support the diagnosis
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of an aggressive neoplasm.
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First, the enhancement is intense.
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Even though it's not dynamic,
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it's an equilibrium-phase enhancement.
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There's a lot of enhancement.
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Then you look at the lesion,
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and it is very heterogeneous inside.
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In fact, when you look very carefully
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at it, it's very heavily septated,
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which plays into the differential diagnosis.
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Next, it has incredible disrespect for boundaries.
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It's even disrespecting the skin.
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It's growing into the dermis.
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If you look at the T1-weighted image and the
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contrast-enhanced image, it's crossing into
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the volar or plantar aspect of the foot.
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So that too suggests an aggressive process.
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So, the differential diagnosis here has to
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include a large complex primary soft
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tissue neoplasm, and the one that would
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work the best here is synovial sarcoma.
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Now there is a differential diagnosis.
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You can get
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synovial chondromas, but they are small.
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You can get an extra-articular soft
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tissue osteogenic sarcoma, but they like
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the upper extremities, they like the
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thigh, and they like the buttock region,
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so this is not a good place for that.
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Soft tissue fibrous sarcoma.
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They're not going to be as heterogeneous
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or as bright on the T2-weighted image,
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so the T2-weighted image is very helpful here.
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One lesion that might be tough to
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differentiate from this lesion,
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but doesn't necessarily like the foot,
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is soft tissue hemangiopericytoma.
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Malignant fibrous histiocytoma,
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it doesn't really have a predilection for
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the foot, it really likes the thigh.
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Whereas this lesion, synovial sarcoma,
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which is the answer, likes the knee and the foot,
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and occasionally will occur in the thigh.
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Giant tophus.
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Well, you'd like to see some erosions
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and some signs of gout, but this would be
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the biggest tophus that I've ever seen.
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And tophagous gout doesn't
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enhance like this, so that is out.
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Myositis ossificans can look really ugly and
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really aggressive, but you get the zonule
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phenomenon, where you get enhancement around
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the outside, and then progressively over time,
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the enhancement may encroach into the center.
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So that's out as well.
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And then you have tumoral calcinosis,
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where you have calcification in the center and
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a bursa-like structure around the outside.
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And on laboratory evaluation, you have
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an abnormal calcium phosphorus product.
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Sometimes they have renal insufficiency.
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So this is synovial sarcoma.
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Now you hear the word synovium.
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There is a benign synovioma of
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the knee joint or of the joint.
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That is not the same lesion.
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It is non-aggressive,
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it is benign, and they're extremely rare.
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These lesions tend to occur in the soft tissues.
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They like to be near tendons,
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tendon sheaths, bursa, less commonly fascia,
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and aponeuroses and the interosseous membrane.
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Again, they're extra-articular,
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which belies their name.
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And, as said, they have a predilection
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for the knee and the foot.
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The average age is about 35 years of age,
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and they tend to calcify about 25% of the time.
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Now, when they calcify heavily,
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they have a better prognosis.
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But for the most part, this lesion
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does not have a great prognosis.
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It does metastasize to the lung.
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And even the lung lesions may calcify.
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Despite its large size, one weird
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aspect about this lesion is it doesn't
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really eat away at bone very much.
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Now because it's composed of epithelium and
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fibrous septa, you get this glandular-like
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appearance with this heavy septation, although
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it's not the organized, brain-like, collagenous,
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parallel septations that you see with very
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aggressive, large, giant, desmoid-like lesions,
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including plantar fibromatosis, which is something
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that occurs in the plantar fascia most commonly.
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About 90% of these have
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translocation of chromosome X to 18.
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And it is believed that these arise from
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pluripotential stem cells from the limb bud.
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There are a few types of these,
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and these include the biphasic type, in which
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you have fibrous and glandular tissue.
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That's this type right here.
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Then monophasic, where either
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fibrous or glandular tissue dominate.
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Those are much less common.
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You have the poorly differentiated type which
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very difficult to differentiate from any other
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poorly differentiated sarcoma other than location.
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You have pure glandular types and then
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you have the heavily calcified type
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which has a pretty good prognosis.
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There is some histologic similarity
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to hemangiopericytoma which can
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make this diagnosis very difficult.
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So, we have some 3D images here that we acquired
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to map out this lesion for potential resection.
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Here is the short axis, the sagittal
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long axis, and the coronal long axis.
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And the lesion has this
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necrotic-looking center to it.
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And you can see its intimate
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contact with the adjacent bone.
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Then we reconstructed it sagittally.
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We reconstructed it in the axial projection.
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It's a nasty lesion.
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It's extra-articular, it likes the foot,
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it likes the knee, it has septations
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and glandular components to it.
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It is the synovial sarcoma.
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Dr. P out.
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