Desmoplastic small round cell tumour: Difference between revisions

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#redirect [[Soft_tissue_lesions#Desmoplastic_small_round_cell_tumour]]
{{ 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.


==General==
*Males > females.
*Usu. affects young adults.
*Typically retroperitoneal.
*Poor prognosis.
==Clinics==
*Rare cases in the CNS reported.
==Microscopic==
Features:<ref name=pmid10207460>{{cite journal |author=Pickhardt PJ, Fisher AJ, Balfe DM, Dehner LP, Huettner PC |title=Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation |journal=Radiology |volume=210 |issue=3 |pages=633–8 |year=1999 |month=March |pmid=10207460 |doi= |url=http://radiology.rsna.org/content/210/3/633.full}}</ref>
#Broad bands of paucicellular fibrous stroma with:
#Small round cells in nests with an undulating sharp border.
#*The small round cells lack distinct nucleoli and have scant cytoplasm; they are ''[[small round cell tumour]]'' cells.
Notes:
*Usu. abundant mitoses.
*+/-Necrosis.
DDx:
*Metastatic [[germ cell tumour]] (DDx of location and age).
*[[Embryonal RMS]].
**It should be noted that DSRCT, like embryonal RMS, is +ve for desmin!
*Solid variant of [[alveolar RMS]].
**Nests in alveolar RMS have round edges.
*Other [[small round cell tumours]].
===Images===
<gallery>
Image:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg | DSRCT - intermed. mag. (WC/Nephron)
Image:Desmoplastic_small_round_cell_tumour_-_high_mag.jpg | DSRCT - high mag. (WC/Nephron)
Image:Desmoplastic_small_round_cell_tumour_-_very_high_mag.jpg | DSRCT - very high mag. (WC/Nephron)
</gallery>
==IHC==
Features:
*[[AE1/AE3]] +ve.
*CAM5.2 usu -ve.
*Desmin +ve (strongly).
*[[EMA]] +ve.
*Actin -ve.
*[[WT-1]] (N-terminal) -ve.
*[[WT-1]] (C-terminal) +ve (nuclear staining).
*CD57 +ve.
*[[Synaptophysin]] focal, patchy +ve.
==Molecular==
*t(11;22)(p13;q12).<ref name=pmid17964965>{{cite journal |author=Lee YS, Hsiao CH |title=Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients |journal=J. Formos. Med. Assoc. |volume=106 |issue=10 |pages=854–60 |year=2007 |month=October |pmid=17964965 |doi=10.1016/S0929-6646(08)60051-0 |url=}}</ref><ref>{{cite journal |author=Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP |title=Results of multimodal treatment for desmoplastic small round cell tumors |journal=J. Pediatr. Surg. |volume=40 |issue=1 |pages=251–5 |year=2005 |month=January |pmid=15868593 |doi=10.1016/j.jpedsurg.2004.09.046 |url=http://www.dsrct.com/JPS%20Article.pdf}}</ref>
==See also==
*[[Soft tissue lesions]].
*[[Small round cell tumours]].
*[[Stromal desmoplasia]].
==References==
{{Reflist|2}}
[[Category:Soft tissue lesions]]
[[Category:Neuropathology]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
Retrieved from "https://librepathology.org/wiki/Desmoplastic_small_round_cell_tumour"