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Following the recent report of an <jats:italic>NR1D1::MAML1<\/jats:italic> gene fusion in an undifferentiated pediatric neoplasm, we performed a retrospective archival review and identified four additional cases of undifferentiated mesenchymal neoplasms with <jats:italic>NR1D1<\/jats:italic>\u2010rearrangement. All four tumors occurred in adult women. The tumors involved superficial and\/or deep soft tissues of the extremities or abdomen. Morphologically, they showed a spectrum of overlapping features. In addition to epithelioid cells, two cases also had a prominent spindle cell component. Two cases also had admixed polygonal cells containing prominent cytoplasmic vacuoles with amorphous debris. The immunophenotype was nonspecific but all cases had at least focal keratin expression; this was extensive in two tumors. Targeted RNA\u2010sequencing revealed two cases each with <jats:italic>NR1D1::MAML1<\/jats:italic> and <jats:italic>NR1D1::MAML2<\/jats:italic> gene fusions. One patient developed lung and liver metastases, and one patient required amputation due to multifocal disease and underlying bone involvement. This study confirms undifferentiated <jats:italic>NR1D1<\/jats:italic>\u2010rearranged sarcoma represents a distinct mesenchymal neoplasm with an epithelioid morphology and potential for aggressive behavior. Further, we offer new insight into the spectrum of clinical, morphologic, immunohistochemical, and molecular findings possible in these rare neoplasms. An awareness of this entity is especially important given the potential for misclassification as a carcinoma.<\/jats:p>","DOI":"10.1002\/gcc.23032","type":"journal-article","created":{"date-parts":[[2022,2,15]],"date-time":"2022-02-15T18:03:30Z","timestamp":1644948210000},"page":"420-426","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Expanding the spectrum of mesenchymal neoplasms with <i>NR1D1<\/i>\u2010rearrangement"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1461-4532","authenticated-orcid":false,"given":"Maribel D.","family":"Lacambra","sequence":"first","affiliation":[{"name":"Department of Anatomic and Cellular Pathology Prince of Wales Hospital, The Chinese University of Hong Kong  Sha Tin Hong Kong China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9717-8205","authenticated-orcid":false,"given":"Cristina 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