How is pleural desmoplastic small round cell tumor (DSRCT) differentiated from Ewing Sarcoma?

Updated: Dec 25, 2019
  • Author: Joseph F Tomashefski, Jr, MD; more...
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The major histologic differential diagnosis of DSRCT is EWS/PNET. The differential diagnoses also include other “small round blue cell tumors” such as small cell carcinoma, small cell variant of malignant mesothelioma, lymphoma, rhabdomyosarcoma, and small cell melanoma. [67]

EWS/PNET is characteristically positive for CD99 and vimentin but negative for cytokeratin, desmin, and WT1. The prototypic chromosomal translocation in EWS/PNET is t(11;22)(q24;q12), which involves the long arm of chromosome 11, in contrast to the translocation observed in DSRCT, which involves the short arm of chromosome 11. [67]

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