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Desmoplastic small round cell tumour (view source)
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'''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. | |||
==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. | |||
===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. | |||
*Desmin +ve. | |||
*EMA +ve. | |||
*Actin -ve. | |||
*WT1 (N-terminal) -ve. | |||
*WT1 (C-terminal) +ve. | |||
*CD57 +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]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Soft tissue lesions]] | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||