Desmoplastic small round cell tumor of the kidney: a case report

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Ertoy Baydar D., Armutlu A., Aydin O., Dağdemir A., Yakupoğlu Y. K.

DIAGNOSTIC PATHOLOGY, vol.15, no.1, 2020 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 15 Issue: 1
  • Publication Date: 2020
  • Doi Number: 10.1186/s13000-020-01015-w
  • Journal Name: DIAGNOSTIC PATHOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, Agricultural & Environmental Science Database, Aquatic Science & Fisheries Abstracts (ASFA), Biotechnology Research Abstracts, EMBASE, MEDLINE, Directory of Open Access Journals
  • Keywords: Desmoplastic small round cell tumor, Kidney, WT1, EWSR1, Case report, FUSION
  • Ondokuz Mayıs University Affiliated: Yes

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.