Summary

Desmoplastic small round cell tumor is a rare and recently described tumor. It has been reported the positivite reaction with epithelial, mesenchymal and neural markers. In this study, we reported three desmoplastic small round cell tumor cases that have follow-up and performed relatively large immunohistochemical panel. Cytokeratin, EMA, desmin, vimentin, NSE and CD15 were positive in each three cases. Synaptophysin (2/3 cases), CD99 (2/3), CD57 (2/3), smooth muscle actin (1/3), Ber-Ep4 (1/3), CA-125 (1/3) were positive in some of the cases and actin was negative. We think that, cytokeratin EMA, desmin, vimentin, NSE and CD15 are useful markers in the diagnosis of desmoplastic small round cell tumor. Of the cases two were died of their disease after 12 months from initial diagnosis and one was alive with disease after 30 months from initial diagnosis reflecting the aggressive clinical course of desmoplastic small round cell tumor.