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Question:
Based on the initial images only, what is your first impression?
Based on the initial images only, what is your first impression?
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CD99
Question
What are your feelings now?
What are your feelings now?
Cam 5.2 and EMA
CD56 and CD57
NSE
Desmin
Negative stains:
WT-1
https://abs.twimg.com/emoji/v2/... draggable="false" alt="❌" title="Kreuzzeichen" aria-label="Emoji: Kreuzzeichen">
Myogeninhttps://abs.twimg.com/emoji/v2/... draggable="false" alt="❌" title="Kreuzzeichen" aria-label="Emoji: Kreuzzeichen">
WT-1
Myogenin
Question:
What is your final diagnosis?
*Desmoplastic small round cell tumour
What is your final diagnosis?
*Desmoplastic small round cell tumour
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#BSTpath
Question:
In males, DSRCTs are more commonly found in the?
In males, DSRCTs are more commonly found in the?
1/ This aggressive tumour classically occurs in the pelvis, mainly males and occasionally females (mean age 20 years).
2/ Paratesticular tumours are rarer and testicular primaries even more so. https://pubmed.ncbi.nlm.nih.gov/9042290/ ">https://pubmed.ncbi.nlm.nih.gov/9042290/&...
2/ Paratesticular tumours are rarer and testicular primaries even more so. https://pubmed.ncbi.nlm.nih.gov/9042290/ ">https://pubmed.ncbi.nlm.nih.gov/9042290/&...
3/ This was difficult from a clinical point of view due to the presentation as a suspected primary germ cell tumour.
4/ Below is ahttps://abs.twimg.com/emoji/v2/... draggable="false" alt="⚠️" title="Warnsignal" aria-label="Emoji: Warnsignal">pre-printhttps://abs.twimg.com/emoji/v2/... draggable="false" alt="⚠️" title="Warnsignal" aria-label="Emoji: Warnsignal"> article discussing primary DSRCT of the testis. https://www.authorea.com/users/366445/articles/486209-a-testicular-and-paratesticular-localization-of-desmoplastic-small-round-cell-tumor-is-it-a-good-prognostic-factor">https://www.authorea.com/users/366...
4/ Below is a
Question:
Which of the following immunohistochemical stains is often positive in these tumours?
Which of the following immunohistochemical stains is often positive in these tumours?
5/ The tumour shows multiphenotypic differentiation, i.e. it expresses
https://abs.twimg.com/emoji/v2/... draggable="false" alt="🧠" title="Gehirn" aria-label="Emoji: Gehirn">Neural
https://abs.twimg.com/emoji/v2/... draggable="false" alt="✅" title="Fettes weißes Häkchen" aria-label="Emoji: Fettes weißes Häkchen">CD56, CD57, NSE
https://abs.twimg.com/emoji/v2/... draggable="false" alt="💪" title="Angespannter Bizeps" aria-label="Emoji: Angespannter Bizeps">Muscle
https://abs.twimg.com/emoji/v2/... draggable="false" alt="✅" title="Fettes weißes Häkchen" aria-label="Emoji: Fettes weißes Häkchen">Desmin (https://abs.twimg.com/emoji/v2/... draggable="false" alt="❌" title="Kreuzzeichen" aria-label="Emoji: Kreuzzeichen">myogenin or MYOD1)
https://abs.twimg.com/emoji/v2/... draggable="false" alt="✍️" title="Schreibende Hand" aria-label="Emoji: Schreibende Hand">Epithelial markers
https://abs.twimg.com/emoji/v2/... draggable="false" alt="✅" title="Fettes weißes Häkchen" aria-label="Emoji: Fettes weißes Häkchen">Keratins, EMA, Ber-EP4
6/ The below article discusses the above. https://journals.lww.com/ajsp/Fulltext/1998/11000/Desmoplastic_Small_Round_Cell_Tumor__II__An.2.aspx">https://journals.lww.com/ajsp/Full...
6/ The below article discusses the above. https://journals.lww.com/ajsp/Fulltext/1998/11000/Desmoplastic_Small_Round_Cell_Tumor__II__An.2.aspx">https://journals.lww.com/ajsp/Full...
Question:
What translocation does this tumour classically harbour?
What translocation does this tumour classically harbour?
7/ The most common translocation is EWSR1-WT1 (t(11;22)(p13;q12).
8/ Cases with ERG and FLI1 have been reported, but these may be variants of Ewings?? https://journals.lww.com/ajsp/Abstract/1992/04000/A_Novel_Reciprocal_Chromosome_Translocation.10.aspx">https://journals.lww.com/ajsp/Abst...
8/ Cases with ERG and FLI1 have been reported, but these may be variants of Ewings?? https://journals.lww.com/ajsp/Abstract/1992/04000/A_Novel_Reciprocal_Chromosome_Translocation.10.aspx">https://journals.lww.com/ajsp/Abst...
Question:
What does the EWSR1-WT1 translocation do?
*platelet-derived growth factor A
**transforming growth factor-β
What does the EWSR1-WT1 translocation do?
*platelet-derived growth factor A
**transforming growth factor-β
9/ Because of the fusion, WT1 is inactivated
10/ WT1 normally represses PDGF-A + TGF- β (+ others)
11/ Although PDGF-A was thought to be the cause of desmoplasia in these tumours, the below study shows the opposite (i.e. the mechanism is more complex) https://www.nature.com/articles/3800264">https://www.nature.com/articles/...
10/ WT1 normally represses PDGF-A + TGF- β (+ others)
11/ Although PDGF-A was thought to be the cause of desmoplasia in these tumours, the below study shows the opposite (i.e. the mechanism is more complex) https://www.nature.com/articles/3800264">https://www.nature.com/articles/...
Last question:
WT-1 immunohistochemistry is positive in?
WT-1 immunohistochemistry is positive in?
12a/ WT-1 immunohistochemistry should show positive nuclear expression however, only to the C-terminus antibody.
12b/ Because of the fusion, in this tumour, a protein containing the zinc finger region of WT1 is only targeted by the C terminus (not N)
https://www.nature.com/articles/3880656.pdf?origin=ppub">https://www.nature.com/articles/...
12b/ Because of the fusion, in this tumour, a protein containing the zinc finger region of WT1 is only targeted by the C terminus (not N)
https://www.nature.com/articles/3880656.pdf?origin=ppub">https://www.nature.com/articles/...
