Abstract
BackgroundDesmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm seen in children and young adults, usually manifested by involvement of abdominal serosa. Here, we present an unusual case of primary DSRCT of kidney.Case presentationThe patient was an 8-year-old girl with a large renal mass which was confused with primitive neuroectodermal tumor (PNET) in the needle biopsy. The tumor had a variegated histology revealing frequent pseudo-rosette formations, pseudopapillary architecture, rhabdoid, clear or pleomorphic cells in addition to typical small round cell morphology and desmoplasia. It showed immunohistochemical features of DSRCT, and EWSR1 re-arrangement.ConclusionsProffering this diagnosis is particularly difficult for tumors of viscera because of the incognizance of the entity in these locations. Moreover, DSRCT is a great mimicker and may get easily confused with more common kidney malignancies of childhood such as Wilms tumor, PNET/EWS, rhabdoid tumor, clear cell sarcoma, and other small round cell tumors as well as renal cell carcinomas. The distinction is critical as the accurate therapeutic approach will require correct diagnosis.
Highlights
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm seen in children and young adults, usually manifested by involvement of abdominal serosa
DSRCT shows a specific reciprocal chromosomal translocation, t(11;22)(p13;q12) (EWSR1WT1 fusion) which generates a chimerical protein with transcriptional regulatory activity
DSRCT primary of the kidney was first described by Su, et al [4] in 2004 and since only a total of 12 cases have been reported in the literature (Table 1) [5–12]
Summary
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm seen in children and young adults, usually manifested by involvement of abdominal serosa. We present an unusual case of primary DSRCT of kidney. The tumor had a variegated histology revealing frequent pseudo-rosette formations, pseudopapillary architecture, rhabdoid, clear or pleomorphic cells in addition to typical small round cell morphology and desmoplasia. It showed immunohistochemical features of DSRCT, and EWSR1 rearrangement. Desmoplastic small round cell tumor (DSRCT) is a rare, distinct entity that was first described by Gerald and Rosai in 1989 [1]. The pathologic diagnosis of this entity can be markedly challenging when it develops in visceral organs such as kidney and especially if diverse and confounding microscopic features are present
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