{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T16:37:31Z","timestamp":1790699851051,"version":"4.1.0"},"reference-count":14,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"20","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCO"],"published-print":{"date-parts":[[2004,10,15]]},"abstract":"<jats:sec><jats:title>Purpose<\/jats:title><jats:p> A balanced chromosomal translocation, t(15;19), resulting in the BRD4-NUT oncogene, has been identified in a lethal carcinoma of young people, a disease described primarily in case reports. We sought to amass a more definitive series of tumors with NUT and\/or BRD4 gene rearrangements and to determine distinct clinicopathologic features. <\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods<\/jats:title><jats:p> Carcinomas (N = 98) in young individuals (median age, 32.5 years) were screened for NUT and BRD4 rearrangements using dual-color fluorescence in situ hybridization. Four published carcinomas with BRD4 and NUT rearrangements were also evaluated. Immunophenotypic analyses were performed. <\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p> Eleven tumors had NUT gene rearrangements, including eight with BRD4-NUT fusions and three with novel rearrangements, which were designated as NUT variant. All NUT-rearranged carcinomas (NRCs) arose from midline epithelial structures, including the first example arising below the diaphragm. Patients were young (median age, 17.6 years). Squamous differentiation (seen in 82% of NRCs) was particularly striking in NUT-variant cases. In this first description of NUT-variant carcinomas, the average survival (96 weeks, n = 3) was longer than for BRD4-NUT carcinomas (28 weeks, n = 8). Strong CD34 expression was found in six of 11 NRCs but in zero of 45 NUT wild-type carcinomas. <\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p> NRCs arise from midline structures in young people, and NRCs with BRD4-NUT are highly lethal, despite intensive therapies. NUT-variant carcinomas might have a less fulminant clinical course than those with BRD4-NUT fusions. CD34 expression is characteristic in NRCs and, therefore, holds promise as a diagnostic test for this distinctive clinicopathologic entity. <\/jats:p><\/jats:sec>","DOI":"10.1200\/jco.2004.02.107","type":"journal-article","created":{"date-parts":[[2004,10,13]],"date-time":"2004-10-13T03:02:45Z","timestamp":1097636565000},"page":"4135-4139","source":"Crossref","is-referenced-by-count":380,"title":["Midline Carcinoma of Children and Young Adults With <i>NUT<\/i> Rearrangement"],"prefix":"10.1200","volume":"22","author":[{"given":"Christopher A.","family":"French","sequence":"first","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffery L.","family":"Kutok","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William C.","family":"Faquin","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey A.","family":"Toretsky","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina R.","family":"Antonescu","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constance A.","family":"Griffin","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vania","family":"Nose","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara O.","family":"Vargas","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary","family":"Moschovi","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fotini","family":"Tzortzatou-Stathopoulou","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isao","family":"Miyoshi","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio R.","family":"Perez-Atayde","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jon C.","family":"Aster","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan A.","family":"Fletcher","sequence":"additional","affiliation":[{"name":"From the Department of Pathology, Brigham and Women\u2019s Hospital; Department of Pathology, Massachusetts General Hospital; Department of Pathology, Children\u2019s Hospital; and Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA; Department of Pediatrics, University of Maryland School of Medicine; and Departments of Pathology and Oncology, The Johns Hopkins University School of Medicine, Baltimore, MD; Department of Pathology, Memorial Sloan-Kettering Cancer..."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"233","reference":[{"key":"R1","unstructured":"Kubonishi I, Takehara N, Iwata J, et al: Novel t(15;19)(q15;p13) chromosome abnormality in a thymic carcinoma. 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