Although the immunohistochemical analysis of classic cases of DSRCT tends to reveal WT1 positivity, N- and C-terminals may be useful as a form of “molecular immunohistochemistry” to identify the EWS–WT1 transcript, as the immunostaining pattern may be altered by variant transcripts and WT1 immunostaining may be negative (as in our case) [35–37]. This evidence concerns the gene WT1 and desmoplastic small round cell tumor.