2014
DOI: 10.1002/gcc.22215
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A genetic dichotomy between pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low‐grade fibromyxoid sarcoma: A pathologic and molecular study of 18 cases

Abstract: Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue tumor exhibiting considerable morphologic overlap with low grade fibromyxoid sarcoma (LGFMS). Moreover, both SEF and LGFMS show MUC4 expression by immunohistochemistry. While the majority of LGFMS cases are characterized by a FUS-CREB3L1 fusion, both FUS-CREB3L2 and EWSR1-CREB3L1 fusions were recently demonstrated in a small number of LGFMS and SEF/LGFMS hybrid tumors. In contrast, recent studies pointed out that SEF harbor frequent EWSR1 rearrang… Show more

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Cited by 96 publications
(92 citation statements)
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(105 reference statements)
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“…Histopathologic features a conglomerate of epithelioid cells arranged in strands, cords, nests, and sheets embedded in a sclerosed and hyalinized stroma [17]. It may resemble infiltrating carcinoma, sclerosing lymphoma, and other sarcomas [4,5], and it may contain areas of conventional fibrosarcoma or minor areas resembling a lowgrade fibromixosarcoma, with transition to higher-grade fibrosarcoma, especially in recurrent cases [18].…”
Section: Discussionmentioning
confidence: 99%
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“…Histopathologic features a conglomerate of epithelioid cells arranged in strands, cords, nests, and sheets embedded in a sclerosed and hyalinized stroma [17]. It may resemble infiltrating carcinoma, sclerosing lymphoma, and other sarcomas [4,5], and it may contain areas of conventional fibrosarcoma or minor areas resembling a lowgrade fibromixosarcoma, with transition to higher-grade fibrosarcoma, especially in recurrent cases [18].…”
Section: Discussionmentioning
confidence: 99%
“…This is particularly difficult to the diagnosis on visceral organs because of its rarity and its epithelioid appearance, closely mimicking carcinomas [18]. A strong and diffuse cytoplasmic reactivity for vimentin is a characteristic feature of sclerosing epithelioid fibrosarcoma, and 70% of the cases show MUC4 positivity [7,14,16,17]. It lacks immunoreactivity for epithelial membrane antigen; cytokeratins (CAM5.2 or AE1/3), HMB-45, neuronspecific enolase, desmin, smooth muscle actin, or muscle common actin (HHF-35) [8] Focal and weak immunostaining for EMA, S-100 protein and more rarely for cytokeratins may be seen in a minority of cases [19].…”
Section: Discussionmentioning
confidence: 99%
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