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Although recurrent\n                    <jats:italic>EWSR1::ATF1<\/jats:italic>\n                    fusions were initially limited to a triad of mesenchymal neoplasms including clear cell sarcoma of soft tissue, angiomatoid fibrous histiocytoma and malignant gastrointestinal neuroectodermal tumor (MGNET), this family has been expanding. We herein describe 4 unclassified extra-abdominal soft tissue (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20093) and bone (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20091) neoplasms displaying epithelioid and round cell morphology and carrying an\n                    <jats:italic>EWSR1::ATF1<\/jats:italic>\n                    fusion. Affected were 3 males and 1 female aged 20\u201356\u00a0years. All primary tumors were extra-abdominal and deep-seated (chest wall, mediastinum, deltoid, and parapharyngeal soft tissue). Their size ranged 4.4\u20137.5\u00a0cm (median, 6.2). One patient presented with constitutional symptoms. Surgery with (2) or without (1) neo\/adjuvant therapy was the treatment. At last follow-up (8\u201321\u00a0months), 2 patients developed progressive disease (1 recurrence; 1 distant metastasis). The immunophenotype of these tumors is potentially misleading with variable expression of EMA (2 of 3), pankeratin (2 of 4), synaptophysin (2 of 3), MUC4 (1 of 3), and ALK (1 of 3). All tumors were negative for S100 and SOX10. These observations point to the existence of heretofore under-recognized group of epithelioid and round cell neoplasms of soft tissue and bone, driven by\n                    <jats:italic>EWSR1::ATF1<\/jats:italic>\n                    fusions, but distinct from established\n                    <jats:italic>EWSR1::ATF1<\/jats:italic>\n                    -associated soft tissue entities. Their overall morphology and immunophenotype recapitulate that of the emerging\n                    <jats:italic>EWSR1\/FUS::CREB<\/jats:italic>\n                    fusion associated intra-abdominal epithelioid\/round cell neoplasms. Our cases point to a potentially aggressive clinical behavior. Recognizing this tumor type is mandatory to delineate any inherent biological and\/or therapeutic distinctness from other, better-known sarcomas in the differential diagnosis including sclerosing epithelioid fibrosarcoma.\n                  <\/jats:p>","DOI":"10.1007\/s00428-024-03879-5","type":"journal-article","created":{"date-parts":[[2024,7,20]],"date-time":"2024-07-20T05:01:44Z","timestamp":1721451704000},"page":"995-1005","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["EWSR1::ATF1 fusions characterize a group of extra-abdominal epithelioid and round cell mesenchymal neoplasms, phenotypically overlapping with sclerosing epithelioid fibrosarcomas, and intra-abdominal FET::CREB fusion neoplasms"],"prefix":"10.1007","volume":"485","author":[{"given":"Bharat","family":"Rekhi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josephine K.","family":"Dermawan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen J.","family":"Fritchie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Zimpfer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tareq M.","family":"Mohammad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatima S.","family":"Ali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koushik","family":"Nandy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youran","family":"Zou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Stoehr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abbas","family":"Agaimy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,7,20]]},"reference":[{"key":"3879_CR1","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1016\/j.path.2018.10.007","volume":"12","author":"K Thway","year":"2019","unstructured":"Thway K, Fisher C (2019) Mesenchymal tumors with EWSR1 gene rearrangements. 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