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LCS is characterized by marked cytologic atypia, frequent mitoses including atypical ones, and expression of CD1a, S100, and langerin (CD207). CD1a and Langerin\u2010positive but S100\u2212 negative LCS is extremely rare in clinical practice. We present a case of a 71\u2010year\u2010old female with a history of melanoma and atypical fibroxanthoma who presented with an erythematous plaque on her left knee. Histopathologic examination revealed a dermal infiltrate comprised of large pleomorphic cells with irregular nuclear contours, prominent longitudinal grooves, and vesicular chromatin, and a high mitotic rate. Notably, there were epidermotropism and a distinctive immunohistochemical profile: S100\u2212, CD1a+, Langerin+, and focal CD68+. Next\u2010generation sequencing identified copy number loss of <jats:italic>CDKN2A<\/jats:italic>, <jats:italic>CDKN2B<\/jats:italic>, and <jats:italic>FOXA1<\/jats:italic>, mutations in <jats:italic>TP53<\/jats:italic>, <jats:italic>POT1<\/jats:italic>, <jats:italic>SH2B3<\/jats:italic>, and <jats:italic>SMARCA4<\/jats:italic>, and a high tumor mutational burden. Herein, we discuss the clinical and pathologic features of 38 cases of LCS with cutaneous involvement reported in the literature, including recent advances in understanding molecular characteristics of this disease. This exceptional case may contribute to our understanding of the etiology of this rare neoplasm.<\/jats:p>","DOI":"10.1111\/cup.14833","type":"journal-article","created":{"date-parts":[[2025,6,24]],"date-time":"2025-06-24T20:24:22Z","timestamp":1750796662000},"page":"554-567","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["An Unusual Case of Cutaneous Langerhans Cell Sarcoma Lacking <scp>S100<\/scp> Expression: A Case Report and Review of the Literature"],"prefix":"10.1111","volume":"52","author":[{"given":"Randa","family":"Obid","sequence":"first","affiliation":[{"name":"University of Louisville  Louisville Kentucky USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Austin R.","family":"Green","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Indiana University  Indianapolis Indiana 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