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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: 12 December 2014, last major update June 2012
Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.

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

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

Sites
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● Usually serosal; rare in kidney

Clinical description
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● Usually presents with advanced stage and poor prognosis

Case reports
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● 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
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● 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
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Peritoneal mass


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

Cytology images
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Site unknown:

Small round blue cells


Cells of variable size and shape with hyperchromatic nuclei

Positive stains
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● 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
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● t(11;22)(p13;q12) with EWS-WT1 fusion transcript

Differential diagnosis
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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


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