TY - JOUR
T1 - Primary renal sclerosing epithelioid fibrosarcoma
T2 - Report of 2 cases with EWSR1-CREB3L1 gene fusion
AU - Argani, Pedram
AU - Lewin, Jack R.
AU - Edmonds, Pamela
AU - Netto, George J.
AU - Prieto-Granada, Carlos
AU - Zhang, Lei
AU - Jungbluth, Achim A.
AU - Antonescu, Cristina R.
N1 - Publisher Copyright:
Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2015/2/28
Y1 - 2015/2/28
N2 - 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.
AB - 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.
KW - fibrosarcoma
KW - renal neoplasm
KW - translocation
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U2 - 10.1097/PAS.0000000000000338
DO - 10.1097/PAS.0000000000000338
M3 - Article
C2 - 25353281
AN - SCOPUS:84923878861
SN - 0147-5185
VL - 39
SP - 365
EP - 373
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 3
ER -