04/26/2010
Metropolis Case1
12 years old girl presented with an ulnar soft tissue swelling, infiltrating
the muscle.
- Case Overview
- Diagnosis
Age:
12
Sex:
female
History:
The ill – circumscribed mass measures 8 x 4.5 x 3.8cm.
Cut surface shows a gray while fleshy appearance.
Cut surface shows a gray while fleshy appearance.
Note:
IHC profile
POSITIVE: BCL-2, Vimentin, CD 99, EMA
NEGATIVE: SMA, DESMIN, LCA, CD 20.
Ki-67: HIGH INDEX.
POSITIVE: BCL-2, Vimentin, CD 99, EMA
NEGATIVE: SMA, DESMIN, LCA, CD 20.
Ki-67: HIGH INDEX.
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Groups:

Comments
Upon reviewing this case again (after 9 months of soft tissue fellowship), I disagree with my previous post. I think this must be a poorly differentiated synovial sarcoma, because, as others point out, there are definitely spindled areas (a feature that is not seen in PNET). The majority of the tumor has a small round cell appearance, a feature which suggests that it is poorly differentiated. I would still do RTPCR or FISH to confirm. Again, excellent case.
Synovial sarcoma for sure. Is study medicine in Romania, Bucharest and help out at a shelter organized by Inchirieri Bucuresti - we mostly deal with car accidents recoveries but time to time something like this appears. I just hate when it's a kid :(
I think I'm correct in saying that Dr Fritz Lang and Dr Mrs Farouk Hutton made the final diagnosis on this, which was: Love Kills.
Some collagen bundles her and there, "ropey"
might be a small cell variety of synovial sarcoma, or undifferentiated SS with strong possibility of EWS/pnet in diff. dg. , what would finally confirm cytogenetic analysis.
Any updates on this one?
SMALL ROUND CELL TUMOUR.NICE PICTURES.
i suggest ewing sarcoma
Is something terrible, not at all what I like reading, but unfortunately there everywhere, even in our Bucuresti was one such case
good case
SARCOMA SINOVIAL VS EWING
Ewings/PNET versus sinovial sarcoma. FISH is appropriate in this case
sarcoma,cell morphpology is of moderate differentiation however the proliferaive index is high
histology not very typical for a synovial cell although ich favors the above diagnosis. cytogenetic is a very helpful thing to do in this case just a comment.
DD:
Ewings/PNET
Synovial sarcoma (but histology is odd)
small cell chondrosarcoma
Very nice
Is the tumour arising from ulna or soft tissue adjacent to ulna? Synovial sarcoma of bone are very rare. Anyways RT-PCR or FISH needs to be done for confirmation.
What about the SYT-SSX transcripts or t(X;18)?
Malignant, high grade, poorly differentiated round cell tumour of soft tissue, possibly Ewing sarcoma, Synovial sarcoma, ..ETC
most cells are small cells.
Ewing's and PNET would be the frontrunners in the differntial diagnosis. I believe, IHC and cytigenetics are mainstay in such cases when the HE is inconclusive.
Fluorescent In Situ Hybridization could be a great help in the diagnosis
We reported it as Poorly differentiated Synovial sarcoma & suggested cytogenetic confirmation. There are definite short spindle cell areas alongwith round cell component & IHC confirms it.
Age does not exclude this diagnosis
IHC & HE suggest Ewings/pnet
IHC profile fits both tumors, as EMA can be positive in some Ewings sarcomas. Age is probably a bit better for Ewings but synovial sarcoma could still occur at this age.
This would be a great case to do FISH looking for t(x;18) for synovial sarcoma and also using a breakapart for EWS gene as a screen for Ewing's sarcoma.
EWS/pnet
solitary fibrous tumor
solitary fibrous tumor
?Small round cell tumour,?EWS/PNET family, Poorly differntiated synovial sarcoma