title: FUS rearrangements are rare in 'pure' sclerosing epithelioid fibrosarcoma.
abstract: Several recent reports have described low-grade fibromyxoid sarcoma with sclerosing epithelioid fibrosarcoma-like areas. We evaluated cases of pure sclerosing epithelioid fibrosarcoma lacking areas of low-grade fibromyxoid sarcoma for FUS rearrangement to determine whether this entity could be related to low-grade fibromyxoid sarcoma. Available formalin-fixed paraffin-embedded tissue of 27 sclerosing epithelioid fibrosarcoma from 25 patients was retrieved and tabulated with clinical information. Unstained slides from formalin-fixed paraffin-embedded blocks were prepared and fluorescence in-situ hybridization was performed using a commercial FUS break-apart probe. The median patient age at presentation was 50 (range, 14-78) years, with 14 males and 10 females. Sclerosing epithelioid fibrosarcoma most commonly involved the extremities (n=8) or chest (n=6). Sixteen patients had a median follow-up of 17 (range, 1-99) months; seven were alive and well at 12 (range, 5-30) months; three alive with disease at 28 (range, 9-99) months; five dead of disease at a median of 22 (range, 1-36) months and one was dead of unknown causes. Twelve patients were known to have metastases; the most common site was lung (n=7), followed by bone (n=3), lymph nodes (n=2) and peritoneum (n=1). Only 2 of 22 (9%) analyzable cases of sclerosing epithelioid fibrosarcoma showed rearrangement in the FUS locus by fluorescence in-situ hybridization. Although cytogenetically confirmed low-grade fibromyxoid sarcoma can have sclerosing epithelioid fibrosarcoma-like areas, FUS rearrangement, which is characteristic of low-grade fibromyxoid sarcoma, appears to be relatively rare in pure sclerosing epithelioid fibrosarcoma.
authors.affiliation: Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
authors.forename: Wei-Lien
authors.initials: WL
authors.lastname: Wang
authors.forename: Harry L
authors.initials: HL
authors.lastname: Evans
authors.forename: Jeanne M
authors.initials: JM
authors.lastname: Meis
authors.forename: Bernadette
authors.initials: B
authors.lastname: Liegl-Atzwanger
authors.forename: Judith V M G
authors.initials: JV
authors.lastname: Bovee
authors.forename: John R
authors.initials: JR
authors.lastname: Goldblum
authors.forename: Steven D
authors.initials: SD
authors.lastname: Billings
authors.forename: Brian P
authors.initials: BP
authors.lastname: Rubin
authors.forename: Dolores
authors.initials: D
authors.lastname: López-Terrada
authors.forename: Alexander J
authors.initials: AJ
authors.lastname: Lazar
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015622:Epithelioid Cells / Q000473:pathology*; D005060:Europe; D005260:Female; D005354:Fibrosarcoma / Q000235:genetics* / Q000401:mortality / Q000556:secondary*; D005404:Fixatives; D005557:Formaldehyde; D015321:Gene Rearrangement; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D016612:Paraffin Embedding; D011379:Prognosis; D034702:RNA-Binding Protein FUS / Q000235:genetics*; D012598:Sclerosis; D013997:Time Factors; D016707:Tissue Fixation / Q000379:methods; D014481:United States; D055815:Young Adult
chemical_list: D005404:Fixatives; D034702:RNA-Binding Protein FUS; D005557:Formaldehyde