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.

中文翻译：

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硬化性上皮样纤维肉瘤：对遗传异质性肿瘤的深入审查

Meis-Kindbloom 等人于 1995 年首次描述。作为纤维肉瘤模拟癌的一种变体，硬化性上皮样纤维肉瘤 (SEF) 是一种恶性软组织肉瘤，其特征在于致密硬化基质中的上皮样细胞、频繁的 MUC4 免疫反应性和具有反复EWSR1基因重排的异质遗传特征。它通常影响偏爱下肢的中年人。据信，SEF 与低级别纤维粘液样肉瘤 (LGFMS) 密切相关，两种肿瘤在形态、免疫表型和分子谱上都表现出重叠的特征。在这篇综述中，我们讨论了 SEF 的临床、形态学和免疫组织化学特征，特别强调其分子多样性和与 LGFMS 的关系。