Desmoplastic small round cell tumour: Difference between revisions

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Desmoplastic_small_round_cell_tumour_-_high_mag.jpg
| Width      =
| Caption    = DSRCT. [[H&E stain]].
| Micro      = small (round) cells in nests with an undulating sharp border, road bands of paucicellular fibrous stroma
| Subtypes  =
| LMDDx      = solid variant of [[alveolar rhabdomyosarcoma]], [[embryonal rhabdomyosarcoma]], other [[small round cell tumours]]
| Stains    =
| IHC        = AE1/AE3 +ve, desmin +ve, EMA +ve, actin -ve, WT1 (N-terminal) -ve, WT1 (C-terminal) +ve, CD57 +ve.
| EM        =
| Molecular  = t(11;22)
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions]] - typically retroperitoneum
| Assdx      =
| Syndromes  =
| Clinicalhx = usu. young adults
| Signs      =
| Symptoms  =
| Prevalence = very rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    = (metastatic) [[germ cell tumour]], [[lymphoma]]
}}
'''Desmoplastic small round cell tumour''', abbreviated '''DSRCT''', is a rare malignant soft tissue lesion that tends to afflict young adults.
'''Desmoplastic small round cell tumour''', abbreviated '''DSRCT''', is a rare malignant soft tissue lesion that tends to afflict young adults.


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*Typically retroperitoneal.
*Typically retroperitoneal.
*Poor prognosis.
*Poor prognosis.
==Clinics==
*Rare cases in the CNS reported.


==Microscopic==
==Microscopic==
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*Solid variant of [[alveolar RMS]].
*Solid variant of [[alveolar RMS]].
**Nests in alveolar RMS have round edges.
**Nests in alveolar RMS have round edges.
*Other [[small round cell tumours]].


===Images===
===Images===
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==IHC==
==IHC==
Features:
Features:
*AE1/AE3 +ve.
*[[AE1/AE3]] +ve.
*Desmin +ve.  
*CAM5.2 usu -ve.
*EMA +ve.
*Desmin +ve (strongly).  
*[[EMA]] +ve.
*Actin -ve.
*Actin -ve.
*WT1 (N-terminal) -ve.
*[[WT-1]] (N-terminal) -ve.
*WT1 (C-terminal) +ve.
*[[WT-1]] (C-terminal) +ve (nuclear staining).
*CD57 +ve.
*CD57 +ve.
*[[Synaptophysin]] focal, patchy +ve.


==Molecular==
==Molecular==
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==See also==
==See also==
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].
*[[Small round cell tumours]].
*[[Stromal desmoplasia]].


==References==
==References==
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[[Category:Soft tissue lesions]]
[[Category:Soft tissue lesions]]
[[Category:Neuropathology]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
Retrieved from "https://librepathology.org/wiki/Desmoplastic_small_round_cell_tumour"