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Here, we describe our experience with mesenchymal mimics of NENs to illustrate their spectrum and draw the attention particularly to a group of mesenchymal\/non-epithelial neoplasms (MN) that combine epithelioid histology with neuroendocrine (NE-) features and peculiar genetic abnormalities. In a consultation series of 4498 cases collected between 2009 and 2021, 2099 neoplasms expressing synaptophysin and\/or chromograninA were reviewed and analyzed. A total of 364 (18%) were diagnosed as non-NENs, while the remaining tumors were NEN. The group of mesenchymal\/non-epithelial neoplasms with NE-features (MN-NE) included 31\/364 (8%) cases. These mostly malignant neoplasms showed an epithelioid morphology. While all but one tumor expressed synaptophysin, mostly patchy, only 10\/29 (34%) co-expressed chromograninA. A total of 13\/31 (42%) of the MN-NE showed <jats:italic>EWSR1<\/jats:italic>-related gene fusions (6 Ewing sarcomas, 5 clear cell sarcomas, and 1 desmoplastic small round cell tumor, 1 neoplasm with <jats:italic>FUS-CREM<\/jats:italic> gene fusion) and 7 (23%) were SWI\/SNF (SMARCB1 or SMARCA4)-deficient neoplasms. The remaining MN-NE included synovial sarcoma, sclerosing epithelioid mesenchymal neoplasm, melanoma, alveolar soft part sarcoma, solitary fibrous tumor, and chordoma. A total of 27\/31 MN-NE were from the last 8\u00a0years, and 6 of them were located in the pancreas. Eleven MN-NE were initially diagnosed as neuroendocrine carcinomas (NECs). MN-NE with epithelioid features play an increasing role as mimickers of NECs. They mostly belong to tumors with gene fusions involving the <jats:italic>EWSR1<\/jats:italic> gene, or with SWI\/SNF complex deficiency. Synaptophysin expression is mostly patchy and chromograninA expression is infrequent in MN-NE of this series and data extracted from literature.<\/jats:p>","DOI":"10.1007\/s00428-021-03156-9","type":"journal-article","created":{"date-parts":[[2021,8,5]],"date-time":"2021-08-05T03:41:08Z","timestamp":1628134868000},"page":"1209-1219","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Mesenchymal\/non-epithelial mimickers of neuroendocrine neoplasms with a focus on fusion gene-associated and SWI\/SNF-deficient tumors"],"prefix":"10.1007","volume":"479","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1130-1744","authenticated-orcid":false,"given":"Atsuko","family":"Kasajima","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bj\u00f6rn","family":"Konukiewitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Melissa","family":"Schlitter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wilko","family":"Weichert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan Hinrich","family":"Br\u00e4sen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abbas","family":"Agaimy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00fcnter","family":"Kl\u00f6ppel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,8,5]]},"reference":[{"key":"3156_CR1","doi-asserted-by":"publisher","first-page":"248","DOI":"10.1038\/modpathol.2014.100","volume":"28","author":"A Agaimy","year":"2015","unstructured":"Agaimy A, Haller F, Frohnauer J, Schaefer IM, Strobel P, Hartmann A, Stoehr R, Kloppel G (2015) Pancreatic undifferentiated rhabdoid carcinoma: KRAS alterations and SMARCB1 expression status define two subtypes. 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