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Among these,\n                    <jats:italic>ZC3H7A\/B::BCOR<\/jats:italic>\n                    sarcomas are less common and are primarily reported as a subset of high-grade endometrial stromal sarcomas, as well as in the spectrum of malignant ossifying fibromyxoid tumors (OFMTs). Herein, we present the clinicopathological, immunohistochemical, and molecular profiles of seven soft tissue tumors exhibiting\n                    <jats:italic>ZC3H7A\/B::BCOR<\/jats:italic>\n                    fusions. The patient\u2019s age\u00a0ranged\u00a0from 13 to 65 years (median\u2009=\u200938). Locations were neck (2) and one case each in the paraspinal region, scalp, gluteal region, chest wall, and thigh. Histologically, the tumors were composed of round to polygonal\u00a0or\u00a0spindle-shaped cells with a variable amount of fibromyxoid stroma, lacking bone shell or ossification, leading to a range of initial differential diagnoses. Immunohistochemically, the tumor cells were positive for S100 (5\/6), cyclin D1 (2\/3), SATB2 (2\/3), BCOR (2\/4), and TLE1 (1\/3) while negative for MUC4 (0\/6), keratin (0\/5), EMA (0\/4), desmin (0\/6), CD34 (0\/6), SMA (0\/5), SOX10 (0\/5), and melanoma cocktail (0\/2). Targeted RNA sequencing revealed\n                    <jats:italic>ZC3H7B::BCOR<\/jats:italic>\n                    fusions in six tumors (four with\n                    <jats:italic>ZC3H7Bex10::BCORex6<\/jats:italic>\n                    and one each\n                    <jats:italic>ZC3H7Bex12::BCORex7<\/jats:italic>\n                    and\n                    <jats:italic>ZC3H7Bex12::BCORex6<\/jats:italic>\n                    ). One tumor revealed a\n                    <jats:italic>ZC3H7Aex10::BCORex6<\/jats:italic>\n                    fusion. All seven tumors were resected, mostly with clear margins (5\/7), including two patients who\u00a0received\u00a0adjuvant therapy. Three of four patients with available follow-up (mean\u2009=\u200945 months) died of disease, while one patient was alive with multiple bone metastases. This series comprises seven additional\n                    <jats:italic>ZC3H7A\/B::BCOR<\/jats:italic>\n                    soft tissue sarcomas associated with aggressive clinical outcomes. Whether this aggressive sarcoma represents a molecular subtype of malignant OFMT or a\u00a0genetic\u00a0variant of\n                    <jats:italic>BCOR<\/jats:italic>\n                    -rearranged sarcomas remains to be further verified.\n                  <\/jats:p>","DOI":"10.1007\/s00428-025-04177-4","type":"journal-article","created":{"date-parts":[[2025,7,24]],"date-time":"2025-07-24T09:51:18Z","timestamp":1753350678000},"page":"1059-1069","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["ZC3H7A\/B::BCOR fusion fibromyxoid sarcoma of soft tissue: an emerging aggressive sarcoma overlapping with malignant ossifying fibromyxoid tumors"],"prefix":"10.1007","volume":"487","author":[{"given":"Bharat","family":"Rekhi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Altan","family":"Kavuncuoglu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nasir Ud","family":"Din","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sameer","family":"Rastogi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali","family":"Abdelsatir","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"}]},{"given":"Kemal","family":"Kosemehmetoglu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,7,24]]},"reference":[{"key":"4177_CR1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1200\/PO.19.00116","volume":"3","author":"NP Mankuzhy","year":"2019","unstructured":"Mankuzhy NP, Anderson B, Kumar C, Heider A, Koschmamn C, Mody RJ (2019) BCOR alterations in pediatric and young adult patients with sarcomas and high-grade glial malignancies: a case series. 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AA is Editor-in-Chief of Virchows Archiv. BR serves on the editorial board of Virchows Archiv.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}