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Non‐fibrosing sclerosing epithelioid fibrosarcoma: an unusual variant
Author(s) -
Puls Florian,
Magnusson Linda,
Niblett Angela,
Douis Hassan,
Peake David,
Taniere Philippe,
Kindblom LarsGunnar,
Mertens Fredrik
Publication year - 2016
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.12791
Subject(s) - university hospital , general hospital , medicine , library science , family medicine , computer science
Sclerosing epithelioid fibrosarcoma (SEF), first described in 1995, is a rare aggressive sarcoma morphologically defined by "epithelioid fibroblasts arranged in distinct cords and nests in a densely sclerotic, hyalinised stroma". SEF can mimic metastatic carcinoma within desmoplastic stroma, lymphoma and other mesenchymal neoplasms such as myoepithelioma, making it difficult to diagnose. In a subset of cases there is morphological overlap between SEF and low grade fibromyxoid sarcoma (LGFMS), a low grade fibroblastic sarcoma. Progression of LGFMS to SEF in single cases is well documented. Recent studies detected EWSR1-CREB3L1 fusion transcripts in a high proportion of SEF. This article is protected by copyright. All rights reserved