Kidney tumor - adult malignancies - Desmoplastic small round cell tumor of kidney

Home Chapter Home Jobs Conferences Fellowships Books

Kidney tumor - adult malignancies

Other neoplasms – adult or adult/child

Desmoplastic small round cell tumor of kidney

Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 30 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
=========================================================================

● Aggressive malignant tumor due to t(11;22)(p13;q12)
● First described in 1989 (Pediatr Pathol 1989;9:177)

Epidemiology
=========================================================================

● Usually male teenagers and young adults, rare in children (Am J Surg Pathol 2007;31:576)

Sites
=========================================================================

● Usually serosal; rare in kidney

Clinical description
=========================================================================

● Usually presents with advanced stage and poor prognosis

Case reports
=========================================================================

● 7 year old girl (J Pediatr Urol 2006;2:52)
● 10 year old boy (Appl Immunohistochem Mol Morphol 2009;17:557)
● 14 year old girl (Pediatr Dev Pathol 2007;10:320)
● 41 year old man (Am J Surg Pathol 2004;28:1379)

Micro 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

Micro images
=========================================================================

Peritoneal mass

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

Cytology images
=========================================================================

Site unknown:

Small round blue cells

Cells of variable size and shape with hyperchromatic nuclei

Positive stains
=========================================================================

● AE1-AE3, desmin (dot like pattern), vimentin (dot like pattern), neuron specific enolase
● Also WT1 (Am J Surg Pathol 2000;24:830), EMA

Molecular 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)

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Desmoplastic small round cell tumor of kidney

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).