{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T20:13:06Z","timestamp":1791231186109,"version":"4.1.0"},"reference-count":104,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T00:00:00Z","timestamp":1661299200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health","award":["AIRC #19975"],"award-info":[{"award-number":["AIRC #19975"]}]},{"name":"Alleanza Contro il Cancro (ACC) Sarcoma WG","award":["AIRC #19975"],"award-info":[{"award-number":["AIRC #19975"]}]},{"name":"Associazione Italiana Ricerca sul Cancro","award":["AIRC #19975"],"award-info":[{"award-number":["AIRC #19975"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Epithelioid sarcoma (ES) is a very rare and aggressive mesenchymal tumor of unclear origin and uncertain lineage characterized by a prevalent epithelioid morphology. The only recurrent genetic alteration reported in ES as yet is the functional inactivation of SMARCB1 (SWI\/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1), a key component of the SWI\/SNF (SWItch\/Sucrose Non-Fermentable) chromatin remodeling complexes. How SMARCB1 deficiency dictates the clinicopathological characteristics of ES and what other molecular defects concur to its malignant progression is still poorly understood. This review summarizes the recent findings about ES pathobiology, including defects in chromatin remodeling and other signaling pathways and their role as therapeutic vulnerabilities.<\/jats:p>","DOI":"10.3390\/cells11172626","type":"journal-article","created":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T21:03:51Z","timestamp":1661375031000},"page":"2626","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Beyond SMARCB1 Loss: Recent Insights into the Pathobiology of Epithelioid Sarcoma"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8917-1353","authenticated-orcid":false,"given":"Elisa","family":"Del Savio","sequence":"first","affiliation":[{"name":"Unit of Oncogenetics and Functional Oncogenomics, Centro di Riferimento Oncologico di Aviano (CRO Aviano) IRCCS, National Cancer Institute, 33081 Aviano, PN, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6642-5592","authenticated-orcid":false,"given":"Roberta","family":"Maestro","sequence":"additional","affiliation":[{"name":"Unit of Oncogenetics and Functional Oncogenomics, Centro di Riferimento Oncologico di Aviano (CRO Aviano) IRCCS, National Cancer Institute, 33081 Aviano, PN, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"v525","DOI":"10.1093\/annonc\/mdx387.013","article-title":"An Epidemiological Insight into Epithelioid Sarcoma (ES): The Open Issue of Distal-Type (DES) versus Proximal-Type (PES)","volume":"28","author":"Frezza","year":"2017","journal-title":"Ann. 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