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The <jats:italic>ING3::PHF1\u2010<\/jats:italic>fusion positive ESS presented in a 58\u2010year\u2010old female as extrauterine mesocolonic, ovarian masses, and displayed large, monomorphic ovoid\u2010to\u2010epithelioid cells arranged in solid sheets. The patient remained alive with disease 13\u2009months after surgery. The three ESS with <jats:italic>EPC1::EED<\/jats:italic> occurred in the uterine corpus in patients with a median age of 58\u2009years (range 27\u201362\u2009years). One tumor showed a uniform epithelioid nested morphology, while the other two were composed of monomorphic spindle cells in fascicles with elevated mitotic figures, focal tumor cell necrosis, and lymphovascular invasion. At a median follow\u2010up of 20\u2009months, two patients developed local recurrence, including one with concomitant distant metastasis, while one patient remained free of disease. All three patients were alive at the last follow\u2010up. The <jats:italic>EPC1::EZH2\u2010<\/jats:italic>fusion positive ESS presented in a 52\u2010year\u2010old female in the uterus, and displayed uniform spindled cells arranged in short fascicles, with focally elevated mitotic activity but without necrosis. The patient remained free of disease 3\u2009months after surgery. All cases were diffusely positive for CD10; four diffusely express estrogen and progesterone receptors. Our study expands the molecular spectrum of <jats:italic>EPC1<\/jats:italic> and <jats:italic>PHF1<\/jats:italic>\u2010related gene fusions in ESS to include additional novel subunits of the PRC2 and\/or NuA4\/TIP60 complexes. These cases displayed a monomorphic epithelioid or spindled phenotype, spanning low\u2010grade and high\u2010grade cytomorphology, all expressing CD10 and commonly ER and PR, and are prone to local and\/or distant spread.<\/jats:p>","DOI":"10.1002\/gcc.23109","type":"journal-article","created":{"date-parts":[[2022,11,29]],"date-time":"2022-11-29T14:49:32Z","timestamp":1669733372000},"page":"152-160","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Expanding the molecular spectrum of gene fusions in endometrial stromal sarcoma: Novel subunits of the chromatin remodeling complexes <scp>PRC2<\/scp> and <scp>NuA4<\/scp>\/<scp>TIP60<\/scp> as alternative fusion partners"],"prefix":"10.1002","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1139-2914","authenticated-orcid":false,"given":"Josephine K.","family":"Dermawan","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory 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