{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T21:20:30Z","timestamp":1778188830265,"version":"3.51.4"},"reference-count":25,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,2,18]],"date-time":"2020-02-18T00:00:00Z","timestamp":1581984000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\"><jats:italic>CIC<\/jats:italic><\/jats:styled-content> rearranged sarcomas have significant overlap with Ewing sarcoma, are aggressive, and typically present in deep soft tissue. They most commonly have a <jats:styled-content style=\"fixed-case\"><jats:italic>t<\/jats:italic><\/jats:styled-content>(4;19)(q35;q13) with <jats:italic><jats:styled-content style=\"fixed-case\">CIC<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DUX4<\/jats:styled-content><\/jats:italic> fusion. Superficial presentation is rare. We report eight (6F, 2M; median 45\u2010years\u2010old, range 14\u201065) superficial <jats:styled-content style=\"fixed-case\"><jats:italic>CIC<\/jats:italic><\/jats:styled-content>\u2010rearranged sarcomas, involving the extremities (n = 4), vulva (n = 2), and trunk (n = 2). The tumors were composed of nodules\/sheets of round cells with necrosis and hemorrhage separated by dense hyaline bands. Tumor cells had vesicular chromatin, prominent nucleoli and frequent mitotic figures. One showed pagetoid spread. Targeted next\u2010generation sequencing was positive for <jats:styled-content style=\"fixed-case\"><jats:italic>CIC\u2010DUX4<\/jats:italic><\/jats:styled-content> fusion (6\/6); fluorescence in situ hybridization (FISH) was positive for <jats:styled-content style=\"fixed-case\"><jats:italic>CIC<\/jats:italic><\/jats:styled-content> rearrangement (2\/3). Eight of eight had evidence of <jats:styled-content style=\"fixed-case\"><jats:italic>CIC\u2010DUX4<\/jats:italic><\/jats:styled-content> fusion\/rearrangement by molecular techniques. Immunohistochemistry was positive for CD99+ (8\/8) and DUX4+ (4\/4). FISH for <jats:styled-content style=\"fixed-case\"><jats:italic>EWSR1<\/jats:italic><\/jats:styled-content> rearrangement was negative (5\/5). Of five patients with at least 6\u2009months follow\u2010up, three of five died of disease, all within 2\u2009years of presentation. One is alive with disease at 48\u2009months. One is disease free at 3\u2009months. Superficial <jats:styled-content style=\"fixed-case\"><jats:italic>CIC<\/jats:italic><\/jats:styled-content>\u2010rearranged sarcomas should be considered in cases exhibiting features reminiscent of Ewing sarcoma, but with increased pleomorphism and\/or geographic necrosis. In contrast to superficial Ewing sarcomas, superficial <jats:styled-content style=\"fixed-case\"><jats:italic>CIC<\/jats:italic><\/jats:styled-content>\u2010rearranged sarcomas are aggressive.<\/jats:p>","DOI":"10.1111\/cup.13656","type":"journal-article","created":{"date-parts":[[2020,2,6]],"date-time":"2020-02-06T04:35:10Z","timestamp":1580963710000},"page":"509-516","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["Superficial sarcomas with <scp><i>CIC<\/i><\/scp> rearrangement are aggressive neoplasms: A series of eight cases"],"prefix":"10.1111","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0629-775X","authenticated-orcid":false,"given":"Jennifer S.","family":"Ko","sequence":"first","affiliation":[{"name":"Pathology and Laboratory Medicine Institute Cleveland Clinic  Cleveland 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Minnesota"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sven","family":"Seiwerth","sequence":"additional","affiliation":[{"name":"Department of Pathology University Hospital Center Zagreb  Zagreb Croatia"},{"name":"Department of Pathology University of Zagreb Medical School  Zagreb Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karen","family":"Fritchie","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1521-4947","authenticated-orcid":false,"given":"Rajiv M.","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Pathology and Clinical Labs, Michigan Medicine University of Michigan  Ann Arbor Michigan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brian 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