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Despite remarkable progress, there are still challenging cases that remain unclassified. In this study, we present an unusual spindle cell sarcoma arising in the abdominal cavity of a 37\u2010year\u2010old female. An extensive panel of immunostains was nonspecific for a line of differentiation and the tumor was subjected to targeted RNA sequencing for further classification. The findings showed a novel <jats:italic>WWTR1::AFF2<\/jats:italic> fusion, which was further confirmed by break\u2010apart FISH analysis for <jats:italic>WWTR1<\/jats:italic> gene rearrangement. The tumor was attached to the wall of sigmoid colon and showed a highly cellular proliferation of plump spindle to epithelioid cells arranged in intersecting fascicles. Areas of extensive endometriosis were identified adjacent to the tumor. The immunoprofile was significant for reactivity with desmin, calponin, WT\u20101, ER, and PR, while negative for CD10, SMA, caldesmon, pan\u2010keratin, ALK, CD117, and S100. The patient is alive and well after 11\u2009months of follow\u2010up. The exact histogenesis of this sarcoma remains unclear, however, the presence of adjacent endometriosis and coexpression of WT1\/ER\/PR raises the possibility of an unusual endometrioid stromal sarcoma, occurring outside the GYN tract. Additional cases are needed to establish the recurrent potential of this fusion event and to better define its pathogenesis and clinical behavior.<\/jats:p>","DOI":"10.1002\/gcc.23045","type":"journal-article","created":{"date-parts":[[2022,4,16]],"date-time":"2022-04-16T09:44:51Z","timestamp":1650102291000},"page":"497-502","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["A novel <i>WWTR1::AFF2<\/i> fusion in an intra\u2010abdominal soft tissue sarcoma with associated endometriosis"],"prefix":"10.1002","volume":"61","author":[{"given":"Nooshin K.","family":"Dashti","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cedar\u2010Sinai  Los Angeles California USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1139-2914","authenticated-orcid":false,"given":"Josephine K.","family":"Dermawan","sequence":"additional","affiliation":[{"name":"Department of Pathology Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. 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