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Sclerosing epithelioid fibrosarcoma: in‐depth review of a genetically heterogeneous tumor

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submitted on 2023-03-15, 08:04 and posted on 2023-03-16, 05:45 authored by Khaled A. Murshed, Issam Al‐Bozom, Adham Ammar

First described in 1995 by Meis-Kindbloom et al. as a variant of fibrosarcoma simulating carcinoma, sclerosing epithelioid fibrosarcoma (SEF) is a malignant soft tissue sarcoma characterized by epithelioid cells in dense sclerotic stroma, frequent immunoreactivity for MUC4 and heterogeneous genetic profile with recurrent EWSR1 gene rearrangement. It typically affects middle-age adults with a predilection for the lower extremity. It is believed that SEF is closely related to low-grade fibromyxoid sarcoma (LGFMS), both tumors show overlapping features in morphology, immunophenotype, and molecular profile. In this review, we discuss the clinical, morphologic, and immunohistochemical features of SEF with particular emphasis on its molecular diversity and relation to LGFMS.

Other Information

Published in: APMIS
License: http://creativecommons.org/licenses/by/4.0/
See article on publisher's website: http://dx.doi.org/10.1111/apm.13157

History

Language

  • English

Publisher

Wiley

Publication Year

  • 2021

Institution affiliated with

  • Hamad Medical Corporation

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