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Desmoplastic Small Round Cell Tumor 20 Years After its Discovery

25 Mar 2015

Abstract excerpt

Desmoplastic small round cell tumor (DSRCT) was proposed as a distinct disease entity by William L Gerald and Juan Rosai in 1991. Over 850 patients have been reported in the medical literature. A specific translocation, t(11;22)(p13;q12), is seen in almost all cases, juxtaposing the EWS gene to the WT1 tumor suppressor gene. DSRCT is composed of nests of small round cells with polyphenotypic differentiation,...

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PMID: 25804122
DOI: 10.2217/fon.15.32

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