Primary renal sclerosing epithelioid fibrosarcoma: report of 2 cases with
EWSR1-CREB3L1 gene fusion
#MMPMID25353281
Argani P
; Lewin JR
; Edmonds P
; Netto GJ
; Prieto-Granada C
; Zhang L
; Jungbluth AA
; Antonescu CR
Am J Surg Pathol
2015[Mar]; 39
(3
): 365-73
PMID25353281
show ga
We report the first 2 genetically confirmed cases of primary renal sclerosing
epithelioid fibrosarcoma (SEF), occurring in a 17-year-old boy and a 61-year-old
woman. In both cases, the tumors demonstrated the typical epithelioid clear cell
morphology associated with extensive hyalinizing fibrosis, raising the
differential diagnosis of solitary fibrous tumor, metanephric stromal tumor, and
the sclerosing variant of clear cell sarcoma of the kidney. Both neoplasms
demonstrated diffuse immunoreactivity for MUC4, a highly specific marker for SEF,
and both demonstrated evidence of rearrangement of both the EWSR1 and CREB3L1
genes, which have recently been shown to be fused in this entity. Both neoplasms
presented with metastatic disease. Primary renal SEF represents yet another
translocation-associated sarcoma now shown to arise primarily in the kidney.
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