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Subcutaneous desmoplastic small round-cell tumor: An unusual primary location expanding the differential of superficial round-cell tumors
Authors:Sepideh N. Asadbeigi  Lisheng Zhang  Konstantinos Linos
Affiliation:1. Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA;2. Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA;3. Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center and Geisel School of Medicine at Dartmouth, One Medical Center Drive, Lebanon, New Hampshire, USA
Abstract:Desmoplastic small round-cell tumor (DSRCT) is a rare, aggressive malignant tumor, which in the great majority of cases arises at abdominal-pelvic sites. Nevertheless, rare cases of primary extra-abdominal tumors have been reported. In challenging cases, its molecular hallmark, the EWSR1-WT1 reciprocal translocation, can be exploited diagnostically by various molecular techniques. Herein, we report an extremely rare case of primary subcutaneous DSRCT in an effort to raise awareness among our dermatopathology colleagues by expanding the differential of superficial round-cell tumors.
Keywords:desmoplastic small round-cell tumor   DSRCT  EWSR1  sarcoma  subcutaneous  WT1
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