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Sclerosing epithelioid fibrosarcoma: in‐depth review of a genetically heterogeneous tumor
Author(s) -
Murshed Khaled A.,
AlBozom Issam,
Ammar Adham
Publication year - 2021
Publication title -
apmis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.909
H-Index - 88
eISSN - 1600-0463
pISSN - 0903-4641
DOI - 10.1111/apm.13157
Subject(s) - pathology , fibrosarcoma , epithelioid sarcoma , sarcoma , stroma , immunophenotyping , biology , immunohistochemistry , soft tissue , medicine , immunology , antigen
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.