Kidney tumor - Adult malignancies
Other neoplasms - Adult or adult / child
Desmoplastic small round cell tumor of kidney

Author: Sean Williamson, M.D. (see Authors page)

Revised: 7 February 2017, last major update June 2012

Copyright: (c) 2003-2017,, Inc.

PubMed Search: Desmoplastic small round cell tumor of kidney

Cite this page: Desmoplastic small round cell tumor of kidney. website. Accessed January 17th, 2018.
Definition / general
  • Usually serosal; rare in kidney
Clinical features
  • Usually presents with advanced stage and poor prognosis
Case reports
Microscopic (histologic) description
  • Nests of small round blue cells of variable size and shape within cellular desmoplastic stroma
  • Tumor cells have scant stroma, indistinct cell borders, hyperchromatic nuclei
  • Frequent mitotic areas and necrotic foci
  • Occasional rosettes; no glandular differentiation
Microscopic (histologic) images

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Image on right: omentum (H&E and INI1+)

Cytology images

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Small round blue cells

Cells with hyperchromatic nuclei

Positive stains
Molecular / cytogenetics description
  • t(11;22)(p13;q12) with EWS-WT1 fusion transcript
Differential diagnosis
  • Ewing's / PNET: no prominent desmoplastic stroma, WT1-, t(11;12) with EWS-FLI1 instead of t(11;22) with EWS-WT1
  • Lymphoma: no prominent desmoplastic stroma, positive for B or T cell markers
  • Neuroblastoma: rosettes, lack of prominent desmoplastic stroma, WT1-
  • Small cell carcinoma: nuclear molding, crush artifact, no prominent desmoplastic stroma; no t(11;22), WT1-
  • Wilms' tumor blastema predominant: usually no nesting pattern, no desmoplastic stroma, no t(11;22)