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=Tumours of uncertain differentiation= | =Tumours of uncertain differentiation= | ||
==Desmoplastic small round cell tumour== | |||
*Abbreviated ''DSRCT''. | |||
===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. | |||
Images: | |||
*[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg DSRCT - intermed. mag. (WC)]. | |||
*[http://en.wikipedia.org/wiki/File:Desmoplastic_small_round_cell_tumour_-_very_high_mag.jpg DSRCT - very high mag. (WC)]. | |||
DDx: | |||
*Metastatic [[germ cell tumour]]. | |||
===IHC=== | |||
Features: | |||
*AE1/AE3 +ve. | |||
*Desmin +ve. | |||
*EMA +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> | |||
==Clear cell sarcoma== | ==Clear cell sarcoma== | ||
*Known among pathologists as "soft-tissue melanoma" and "melanoma of the soft parts", as it has a strong morphological resemblance.<ref name=pmid18300804>{{cite journal |author=Hisaoka M, Ishida T, Kuo TT, ''et al.'' |title=Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases |journal=Am. J. Surg. Pathol. |volume=32 |issue=3 |pages=452–60 |year=2008 |month=March |pmid=18300804 |doi=10.1097/PAS.0b013e31814b18fb |url=}}</ref> | *Known among pathologists as "soft-tissue melanoma" and "melanoma of the soft parts", as it has a strong morphological resemblance.<ref name=pmid18300804>{{cite journal |author=Hisaoka M, Ishida T, Kuo TT, ''et al.'' |title=Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases |journal=Am. J. Surg. Pathol. |volume=32 |issue=3 |pages=452–60 |year=2008 |month=March |pmid=18300804 |doi=10.1097/PAS.0b013e31814b18fb |url=}}</ref> |
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