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We present 14 cases of uni- (<jats:italic>n<\/jats:italic> = 9) or multifocal (<jats:italic>n<\/jats:italic> = 5) undifferentiated large cell\/rhabdoid carcinoma presenting in the bowel of patients with concurrent (<jats:italic>n<\/jats:italic> = 9) or recent (diagnosed 1 to 25 months earlier; median, 4) non-small cell lung cancer (NSCLC). Patients were 6 females and 8 males, aged 52 to 85 years. Primary NSCLC was verified histologically in 10 cases and by imaging in 4. The undifferentiated histology was present in the lung biopsy in 4\/10 patients (as sole pattern in 3 and combined with adenocarcinoma in 1) and was limited to the intestinal metastases in the remainder. PDL1 was strongly expressed in 7\/9 cases (CPS: 41 to 100). Loss of at least one SWI\/SNF subunit was detected in 7\/13 cases (54%). SMARCA2 loss (<jats:italic>n<\/jats:italic> = 6) was most frequent and was combined with SMARCA4 loss in one case. PBRM1 loss was observed in one tumor. Successful molecular testing of 11 cases revealed <jats:italic>BRAF<\/jats:italic> mutations in 4 (3 were non-V600E variants), <jats:italic>KRAS<\/jats:italic> mutations in 3, and wildtype in 4. None had <jats:italic>EGFR<\/jats:italic> mutations. Analysis of 4 paired samples revealed concordant <jats:italic>KRAS<\/jats:italic> (2) and <jats:italic>BRAF<\/jats:italic> (1) mutations or wildtype (1). Our study indicates that undifferentiated carcinoma within the intestines of patients with concurrent\/recent NSCLC represents dedifferentiated metastasis from the NSCLC. Recognition of this unusual presentation is cardinal to avoid misdiagnosis with inappropriate therapeutic and prognostic implications.<\/jats:p>","DOI":"10.1007\/s00428-021-03032-6","type":"journal-article","created":{"date-parts":[[2021,1,27]],"date-time":"2021-01-27T17:03:06Z","timestamp":1611766986000},"page":"157-167","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Undifferentiated large cell\/rhabdoid carcinoma presenting in the intestines of patients with concurrent or recent non-small cell lung cancer (NSCLC): clinicopathologic and molecular analysis of 14 cases indicates an unusual pattern of dedifferentiated metastases"],"prefix":"10.1007","volume":"479","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0445-8161","authenticated-orcid":false,"given":"Abbas","family":"Agaimy","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ondrej","family":"Daum","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michal","family":"Michal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mona W.","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Stoehr","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arndt","family":"Hartmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory Y.","family":"Lauwers","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,1,27]]},"reference":[{"key":"3032_CR1","volume-title":"5th","author":"WHO Classification of Tumours of the Digestive System","year":"2019","unstructured":"WHO Classification of Tumours of the Digestive System (2019) 5th. 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