{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T02:00:14Z","timestamp":1792202414150,"version":"4.4.0"},"reference-count":44,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2017,6,23]],"date-time":"2017-06-23T00:00:00Z","timestamp":1498176000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Nuclear protein of the testis (\n                      <jats:italic>NUT<\/jats:italic>\n                      ) carcinoma (formerly\n                      <jats:italic>NUT<\/jats:italic>\n                      midline carcinoma) is an aggressive tumor defined by the presence of\n                      <jats:italic>NUT<\/jats:italic>\n                      rearrangement with a poor prognosis. This rare cancer is underdiagnosed and poorly treated.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>\n                      The primary objective of this study was to describe the clinical, radiologic, and biological features of\n                      <jats:italic>NUT<\/jats:italic>\n                      carcinoma. The secondary objective was to describe the various treatments and assess their efficacy.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      This retrospective multicenter study was based on review of the medical records of children and adults with\n                      <jats:italic>NUT<\/jats:italic>\n                      carcinoma with specific rearrangement or positive anti\u2010NUT nuclear staining (&gt;50%).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>This series of 12 patients had a median age of 18.1 years (ranges: 12.3\u201349.7 years). The primary tumor was located in the chest in eight patients, the head and neck in three patients, and one patient had a multifocal tumor. Nine patients presented regional lymph node involvement and eight distant metastases. One\u2010half of patients were initially misdiagnosed. Specific NUT antibody was positive in all cases tested. A transient response to chemotherapy was observed in four of 11 patients. Only two patients were treated by surgery and five received radiotherapy with curative intent. At the end of follow\u2010up, only one patient was still in remission more than 12 years after the diagnosis. Median overall survival was 4.7 months (95% confidence interval [CI]: 2.1\u201317.7).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      <jats:italic>NUT<\/jats:italic>\n                      carcinoma is an aggressive disease refractory to conventional therapy. Early diagnosis by NUT\u2010specific antibody immunostaining in cases of undifferentiated or poorly differentiated carcinoma to identify the specific rearrangement of\n                      <jats:italic>NUT<\/jats:italic>\n                      gene is useful to propose the optimal therapeutic strategy.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/pbc.26693","type":"journal-article","created":{"date-parts":[[2017,6,23]],"date-time":"2017-06-23T03:43:58Z","timestamp":1498189438000},"source":"Crossref","is-referenced-by-count":79,"title":["<i>NUT<\/i>\n                    carcinoma in children and adults: A multicenter retrospective study"],"prefix":"10.1002","volume":"64","author":[{"given":"Lauriane","family":"Lemelle","sequence":"first","affiliation":[{"name":"Department of Pediatric Adolescent Young Adults Institut Curie  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ga\u00eblle","family":"Pierron","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de G\u00e9n\u00e9tique somatique Institut Curie  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Fr\u00e9neaux","sequence":"additional","affiliation":[{"name":"D\u00e9partement de pathologie Institut Curie  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Huybrechts","sequence":"additional","affiliation":[{"name":"Hematology\u2010Oncology Unit H\u00f4pital Universitaire des Enfants Reine Fabiola ULB Universit\u00e9 libre de Bruxelles  Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Spiegel","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology CHU Hautepierre  Strasbourg France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Plantaz","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology H\u00f4pital de Grenoble  Grenoble France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morbize","family":"Julieron","sequence":"additional","affiliation":[{"name":"Head and Neck Surgery Department Centre Oscar Lambret  Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Dumoucel","sequence":"additional","affiliation":[{"name":"CHU Sainte Justine University of Montreal  Montreal Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoine","family":"Italiano","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology Institut Bergoni\u00e9  Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9deric","family":"Millot","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Department Centre Hospitalier Universitaire Poitiers  Poitiers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christophe","family":"Le Tourneau","sequence":"additional","affiliation":[{"name":"Medical Oncology Department Institut Curie  Paris France"},{"name":"INSERM U900 Research unit  Saint\u2010Cloud France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guy","family":"Leverger","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology Assistance Publique \u2013 H\u00f4pitaux de Paris H\u00f4pital Armand Trousseau  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Chastagner","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology H\u00f4pital d'Enfants de Brabois  Vandoeuvre Les Nancy France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthieu","family":"Carton","sequence":"additional","affiliation":[{"name":"Population\u2010Based Epidemiological Cohorts Unit INSERM UMS 11  Villejuif France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2520-139X","authenticated-orcid":false,"given":"Daniel","family":"Orbach","sequence":"additional","affiliation":[{"name":"Department of Pediatric Adolescent Young Adults Institut Curie  Paris France"},{"name":"French Pediatric Rare Tumor Group (groupe Fracture)"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,6,23]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199124000-00011"},{"issue":"12","key":"e_1_2_8_3_1","first-page":"3327","article-title":"Novel t(15;19)(q15;p13) chromosome abnormality in a thymic carcinoma","volume":"51","author":"Kubonishi I","year":"1991","journal-title":"Cancer Res"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-12-1153"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-010-0235-x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318198d666"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-pathol-011811-132438"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.30242"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-011-2272-3"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2004.02.107"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181abe120"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-010-0174-6"},{"issue":"6","key":"e_1_2_8_13_1","doi-asserted-by":"crossref","first-page":"481","DOI":"10.2350\/09-10-0727-CR.1","article-title":"NUT midline carcinoma in a newborn with multiorgan disseminated tumor and a 2\u2010year\u2010old with a pancreatic\/hepatic primary","volume":"13","author":"Shehata BM","journal-title":"Pediatr Dev Pathol"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20755"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-10-3513"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1517\/13543776.2014.859244"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/bs.acr.2016.04.001"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25350"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2014.54"},{"key":"e_1_2_8_20_1","first-page":"1","article-title":"Bromodomain and extraterminal protein inhibitors in pediatrics: a review of the literature","volume":"4","author":"Jim\u00e9nez I","year":"2016","journal-title":"Pediatr Blood Cancer"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-15-1335"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1684\/bdc.2010.1169"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2008.10.026"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63049-0"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1097\/JTO.0000000000000630"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13143"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-013-0428-1"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/dc.23291"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(15)00583-5"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31815a3900"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/dc.23506"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1158\/2159-8290.CD-14-0014"},{"issue":"2","key":"e_1_2_8_33_1","first-page":"572951","article-title":"Cytological features of a variant NUT midline carcinoma of the lung harboring the NSD3\u2010NUT fusion gene: a case report and literature review","volume":"2015","author":"Kuroda S","year":"2015","journal-title":"Case Rep Pathol"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1179\/1973947815Y.0000000046"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.12659\/AJCR.893879"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.lungcan.2015.10.001"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature09504"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1158\/1538-7445.AM2016-CT014"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.4081\/rt.2016.6241"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M114.600759"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1038\/emboj.2010.176"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.267583.115"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0032-1327608"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678707.2017.1298440"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1002\/cam4.426"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.26693","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.26693","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T04:54:17Z","timestamp":1695963257000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.26693"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,23]]},"references-count":44,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2017,12]]}},"alternative-id":["10.1002\/pbc.26693"],"URL":"https:\/\/doi.org\/10.1002\/pbc.26693","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,23]]},"article-number":"e26693"}}