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Review
. 2020 Aug;47(8):768-775.
doi: 10.1111/cup.13703. Epub 2020 Jun 2.

Subcutaneous desmoplastic small round-cell tumor: An unusual primary location expanding the differential of superficial round-cell tumors

Sepideh N Asadbeigi  1 , Lisheng Zhang  2 , Konstantinos Linos  3
Affiliations
Review

Subcutaneous desmoplastic small round-cell tumor: An unusual primary location expanding the differential of superficial round-cell tumors

Sepideh N Asadbeigi et al. J Cutan Pathol. 2020 Aug.

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: EWSR1; WT1; desmoplastic small round-cell tumor; DSRCT; sarcoma; subcutaneous.

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