Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Wang, WL; Evans, HL; Meis, JM; Liegl-Atzwanger, B; Bovee, JVMG; Goldblum, JR; Billings, SD; Rubin, BP; Lopez-Terrada, D; Lazar, AJ; .
FUS rearrangements are rare in 'pure' sclerosing epithelioid fibrosarcoma.
Mod Pathol. 2012; 25(6):846-853
Doi: 10.1038/modpathol.2011.214
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- Co-Autor*innen der Med Uni Graz
- Liegl-Atzwanger Bernadette
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Adult -
- Aged -
- Epithelioid Cells - pathology
- Epithelioid Cells -
- Female -
- Fibrosarcoma - genetics
- Fixatives -
- Formaldehyde -
- Gene Rearrangement -
- Humans -
- In Situ Hybridization, Fluorescence -
- Male -
- Middle Aged -
- Neoplasm Grading -
- Paraffin Embedding -
- Prognosis -
- RNA-Binding Protein FUS - genetics
- Sclerosis -
- Time Factors -
- Tissue Fixation - methods
- Tissue Fixation -
- Young Adult -
- Find related publications in this database (Keywords)
- FUS
- low-grade fibromyxoid sarcoma
- sclerosing epithelioid sarcoma