PMID- 34894816 OWN - NLM STAT- MEDLINE DCOM- 20220517 LR - 20220517 IS - 1940-2465 (Electronic) IS - 1066-8969 (Linking) VI - 30 IP - 4 DP - 2022 Jun TI - Primary Sclerosing Epithelioid Fibrosarcoma of the Kidney: A Case Report and Review of the Literature. PG - 437-442 LID - 10.1177/10668969211065107 [doi] AB - Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma. We report one case of primary kidney SEF occurring in a 38-year-old man. Microscopically, epithelioid neoplastic cells are mainly arranged in cords and nests embedded in the dense sclerosing stroma. Diffuse immunohistochemical staining for MUC4 in neoplastic cells and the presence of the EWSR1 gene split by fluorescence in situ hybridization (FISH) analysis confirmed the histological diagnosis. Primary kidney SEF is extremely rare, the differential diagnosis strategy broadly includes a series of tumors with epithelioid morphology and sclerosing matrix, mainly including sclerosing variants of clear cell sarcoma of the kidney (CCSK), renal synovial sarcoma (SS), renal solitary fibrous tumor (SFT), metanephric stromal tumor (MST), sclerosing perivascular epithelioid cell tumor (PEComa), and carcinomas, and immunohistochemical expression of MUC4 and evidence of the EWSR1 gene split are helpful in making a definite diagnosis. FAU - Peng, Yan AU - Peng Y AUID- ORCID: 0000-0001-9972-9640 AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. FAU - Chen, Tongbing AU - Chen T AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. FAU - Zhang, Dachuan AU - Zhang D AUID- ORCID: 0000-0002-9778-3456 AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. FAU - Wu, Chao AU - Wu C AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. FAU - Lei, Ting AU - Lei T AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. FAU - Shi, Yongqiang AU - Shi Y AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. FAU - Xia, Cunyan AU - Xia C AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. FAU - Li, Qing AU - Li Q AD - 117850The Third Affiliated Hospital of Soochow University, Changzhou First People's Hospital, Changzhou City, Jiangsu Province, China. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20211213 PL - United States TA - Int J Surg Pathol JT - International journal of surgical pathology JID - 9314927 RN - 0 (Biomarkers, Tumor) SB - IM MH - Biomarkers, Tumor/genetics/metabolism MH - Epithelioid Cells/pathology MH - Female MH - *Fibrosarcoma/diagnosis/genetics MH - Humans MH - In Situ Hybridization, Fluorescence MH - Kidney/pathology MH - *Kidney Neoplasms/diagnosis/genetics MH - Male OTO - NOTNLM OT - EWSR1 OT - clinicopathologic features OT - immunophenotype OT - kidney OT - sclerosing epithelioid fibrosarcoma EDAT- 2021/12/14 06:00 MHDA- 2022/05/18 06:00 CRDT- 2021/12/13 11:19 PHST- 2021/12/14 06:00 [pubmed] PHST- 2022/05/18 06:00 [medline] PHST- 2021/12/13 11:19 [entrez] AID - 10.1177/10668969211065107 [doi] PST - ppublish SO - Int J Surg Pathol. 2022 Jun;30(4):437-442. doi: 10.1177/10668969211065107. Epub 2021 Dec 13.