Kidney tumor - adult malignancies - Clear cell sarcoma of soft parts

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Kidney tumor - adult malignancies

Other neoplasms – adult or adult/child

Clear cell sarcoma of soft parts - adults

Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 26 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

See also Soft tissue chapter

General
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● Also called melanoma of soft parts
● Very rare in kidney, with only one case report

Case reports
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● 20 year old man with metastases to liver and lung and death 5 years later (Am J Surg Pathol 1999;23:589)

Micro description
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● Nests or short fascicles of spindled or epithelioid cells with clear to granular eosinophilic cytoplasm separated by fibrous septa; surrounds normal epithelial elements
● Also multinucleated giant cells, variable melanin

Positive stains
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● S100, HMB45

Negative stains
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● Keratin, CD99

Molecular description
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● t(12;22)(q13;q12), EWS-ATF1 gene region rearrangement in >95% [at other sites]

Differential diagnosis
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● Clear cell sarcoma: very young children, similar histology but nests are formed by vascular structures, S100-, HMB45-, different translocation
● Wilms’ tumor: usually triphasic, no dominant nesting pattern, negative for melanocytic markers

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Clear cell sarcoma of soft parts - adults

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