title: Sclerosing epithelioid fibrosarcoma.
abstract: We report the case of a 55-year-old woman who developed a mass in the soft tissue of the anterior right proximal thigh. Microscopic findings in the biopsy specimen supported the diagnosis of sclerosing epithelioid fibrosarcoma (SEF). This entity is part of a spectrum of lesions that includes low-grade fibromyxoid sarcoma (LGFMS) and hyalinizing spindle cell tumor with giant rosettes (HSCTGR). It shares with LGFMS occasional overlapping histopathologic features and immunopositivity for MUC4. Although FUS and CREB3L2 gene rearrangements, characteristics of LGFMS, have been found in hybrid tumors (those with features of both SEF and LGFMS) and in examples of LGFMS with relapses showing SEF morphology, it appears that EWSR1 and CREB3L1 rearrangements predominate over FUS and CREB3L2 rearrangements among "pure" SEF tumors. Recent information about SEF is briefly reviewed.
authors.affiliation: Department of Pathology, University of Virginia Health System, 1215 Lee Street, 800214, 22908, Charlottesville, VA, USA.
authors.forename: James W
authors.initials: JW
authors.lastname: Patterson
authors.affiliation: Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior (MVR-Sofia), General Skobelev Nr 79, 1606, Sofia, Bulgaria. georgi_tchernev@yahoo.de.
authors.forename: Georgi
authors.initials: G
authors.lastname: Tchernev
authors.affiliation: "Onkoderma" - Policlinic for Dermatology and Dermatologic Surgery, General Skobelev 26 blvd., Sofia, Bulgaria.|Department of Dermatology and Venereology, Medical University of Plovdiv, Plovdiv, Bulgaria.
authors.forename: Anastasiya Atanasova
authors.initials: AA
authors.lastname: Chokoeva
authors.affiliation: Department of Pathology, University of Virginia Health System, 1215 Lee Street, 800214, 22908, Charlottesville, VA, USA.
authors.forename: Mark R
authors.initials: MR
authors.lastname: Wick
mesh_terms: D001706:Biopsy; D017362:Cyclic AMP Response Element-Binding Protein / Q000235:genetics; D015622:Epithelioid Cells / Q000473:pathology; D005260:Female; D005354:Fibrosarcoma / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D015321:Gene Rearrangement / Q000235:genetics; D006801:Humans; D008875:Middle Aged; D055264:Mucin-4 / Q000235:genetics; D009419:Nerve Tissue Proteins / Q000235:genetics; D034802:RNA-Binding Protein EWS / Q000235:genetics; D034702:RNA-Binding Protein FUS / Q000235:genetics; D012983:Soft Tissue Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D013848:Thigh / Q000473:pathology / Q000601:surgery
chemical_list: C485618:CREB3L1 protein, human; D017362:Cyclic AMP Response Element-Binding Protein; C500986:EWSR1 protein, human; C000599550:FUS protein, human; C524658:MUC4 protein, human; D055264:Mucin-4; D009419:Nerve Tissue Proteins; D034802:RNA-Binding Protein EWS; D034702:RNA-Binding Protein FUS
keywords: CREB3L1; FUS; LGFMS; SEF; Sclerosing