Desmoplastic small round cell tumor DSRCT

The term polyphenotypic small round cell tumor seems most appropriate to describe the general attributes of these lesions. The desmoplastic small round cell tumor (DSRCT) is the best-recognized representative of this group. It is characterized by an EWS/WTl chimeric transcript, and is a morphologically distinctive neoplasm that is characterized by reactive fibrosis surrounding discrete nests of tumor cells (Fig. 4.15). DSRCT has a relatively complicated immunoprofile. 

An antibody to the car boxy-terminal (C-terminal) region of the WT gene (WTl) is useful in recognition of desmoplastic small round cell tumor. DSRCT shows strong nuclear staining with a C-terminal WTl antibody WT and RMS can also show nuclear reactivity for WTl.37,47,48 have observed occasional examples of... 

Fang X, Rodabaugh K, Penetrante R, et al. Desmoplastic small round cell tumor (DSRCT) with ovarian involvement in 2 young women. Appl Immunohistochem Mol Morphol. 2008 16 94-99. 

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