Abstract
We present two cases of sclerosing epithelioid fibrosarcoma (SEF) that arose primarily in the kidney. The tumor in both cases was located at the upper pole of the kidney. Clincially, they were suspected as renal cell carcinomas. However, histological examination revealed densely hyalinized epithelioid tumor suggestive of SEF. The diffuse immunohistochemical staining of MUC4 by neoplastic cells and the presence of EWSR1 gene rearrangement by subsequent florescence in site hybridization (FISH) analysis confirmed the histological diagnosis. Molecular cytogenetic study is highly helpful in arriving at a final diagnosis, in particular to a rare tumor type that arises at an unusual site.
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