{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T17:41:05Z","timestamp":1791222065255,"version":"4.1.0"},"reference-count":14,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2014,3,14]],"date-time":"2014-03-14T00:00:00Z","timestamp":1394755200000},"content-version":"vor","delay-in-days":41,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2014,2]]},"abstract":"<jats:p><jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>hwachman\u2013<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>iamond syndrome, which is characterized by pancreatic fatty degeneration, skeletal growth retardation, and hematological dysfunction, is a congenital disease caused by <jats:italic><jats:styled-content style=\"fixed-case\">SBDS<\/jats:styled-content><\/jats:italic> gene mutations. Although hematological disorders often accompany this syndrome, carcinomas associated with this syndrome have not been reported except in one breast cancer and one moderately differentiated pancreatic cancer case. We report on an autopsy of a 24\u2010year\u2010old case of pancreatoduodenal carcinoma in <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>hwachman\u2013<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>iamond syndrome. The histology of the tumor was undifferentiated carcinoma, which seems to have originated from either the pancreatic duct or the duodenal epithelium. The tumor was intermingled with two pathological changes characteristic of <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>hwachman\u2013<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>iamond syndrome: fatty degeneration of the pancreas and inflammation of the villous stroma of the duodenum. Considering that <jats:styled-content style=\"fixed-case\">SBDS<\/jats:styled-content> protein regulates mitosis and its suppression causes genomic instability, this case might provide an example of carcinogenesis based on genomic instability, together with degenerative changes and chronic inflammation, at a very young age.<\/jats:p>","DOI":"10.1111\/pin.12133","type":"journal-article","created":{"date-parts":[[2014,3,14]],"date-time":"2014-03-14T11:21:58Z","timestamp":1394796118000},"page":"75-80","source":"Crossref","is-referenced-by-count":10,"title":["Young\u2013age\u2010onset pancreatoduodenal carcinoma in <scp>S<\/scp>hwachman\u2013<scp>D<\/scp>iamond syndrome"],"prefix":"10.1111","volume":"64","author":[{"given":"Takeo","family":"Nakaya","sequence":"first","affiliation":[{"name":"Department of Molecular Pathology Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Kurata","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathology Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotsugu","family":"Hashimoto","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathology Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shigeo","family":"Nishimata","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuyo","family":"Kashiwagi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Fujita","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathology Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hisashi","family":"Kawashima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiko","family":"Kuroda","sequence":"additional","affiliation":[{"name":"Department of Molecular Pathology Tokyo Medical University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,3,14]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(64)80150-5"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1964.tb07237.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03585.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1062"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24052"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2010.01468.x"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI33764"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24453"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/tropej\/fml050"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-004-1081-2"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-11-4371"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199305203282001"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000149708.12335.6a"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2010.077677"}],"container-title":["Pathology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpin.12133","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pin.12133","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T19:05:19Z","timestamp":1696014319000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pin.12133"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,2]]},"references-count":14,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2014,2]]}},"alternative-id":["10.1111\/pin.12133"],"URL":"https:\/\/doi.org\/10.1111\/pin.12133","archive":["Portico"],"relation":{},"ISSN":["1320-5463","1440-1827"],"issn-type":[{"value":"1320-5463","type":"print"},{"value":"1440-1827","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,2]]}}}