Agaimy A (2019)
Publication Type: Journal article, Review article
Publication year: 2019
DOI: 10.1007/s00428-019-02677-8
Epithelioid cell features mimicking carcinomas characterize a variety of histogenetically, phenotypically, and molecularly distinct subsets of mesenchymal neoplasms. In a pathogenetic sense, epithelioid soft tissue tumors basically fall into three main genetic categories: (1) switch/sucrose non-fermenting (SWI/SNF) complex-deficient tumors (with epithelioid sarcoma as their prototype); (2) epithelioid neoplasms driven by specific rare gene fusions (such as sclerosing epithelioid fibrosarcoma with EWSR1 fusions and GLI1-related malignant epithelioid soft tissue neoplasms); and (3) a heterogeneous group encompassing epithelioid variants of diverse other entities. Notably, lesions in the first and third groups may display variable, occasionally prominent, rhabdoid cell morphology, thus further complicating their differential diagnosis. This review summarizes the main clinicopathological, phenotypic, and genotypic features of these diseases and discusses their pertinent differential diagnostic considerations.
APA:
Agaimy, A. (2019). What is new in epithelioid soft tissue tumors? Virchows Archiv. https://doi.org/10.1007/s00428-019-02677-8
MLA:
Agaimy, Abbas. "What is new in epithelioid soft tissue tumors?" Virchows Archiv (2019).
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