{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T15:02:34Z","timestamp":1787065354565,"version":"build-2736575974"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T00:00:00Z","timestamp":1592265600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T00:00:00Z","timestamp":1592265600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["surg case rep"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Langerhans cell histiocytosis (LCH) is an abnormal accumulation of Langerhans cells in various organs that sometimes induces organ dysfunction. LCH can affect the liver, resulting in sclerosing cholangitis and biliary cirrhosis. However, liver and bile duct involvement is usually observed in the disseminated form of LCH. We herein report a rare case of LCH localized only in the extrahepatic bile duct that resulted in severe liver cirrhosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 3-year-old boy with elevated liver enzymes, obstructive jaundice, and dilation of the common bile duct was referred to our institution. Contrast-enhanced computed tomography showed atrophy of the right hepatic lobe, relative hypertrophy of the left hepatic lobe, choledocholiths, and biliary debris extensively with biliary duct dilation. Magnetic resonance cholangiopancreatography revealed dilation of the intrahepatic and extrahepatic bile ducts and multiple choleliths in the gallbladder and common bile duct. Laparoscopic cholecystectomy, intraoperative cholangiography, liver biopsy, and gastrointestinal fiberscopy were performed. A liver specimen showed severe biliary cirrhosis due to sclerosing cholangitis. The patient then underwent living-donor liver transplantation because of severe liver cirrhosis 3\u2009months after the first surgery. The common bile duct was not suitable for duct-to-duct anastomosis and was resected because of severe inflammation. Histologic sections of the common bile duct showed histiocytic cell proliferation. Immunohistochemistry revealed histiocytoses that were positive for Langerin, S-100 protein, and CD1a. However, no histiocytic cell proliferation was noted in the liver tissue. The definitive diagnosis was LCH localized to the extrahepatic bile duct. LCH in the extrahepatic bile duct seemed to cause sclerosing cholangitis. The patient was discharged uneventfully 2\u2009months after living-donor liver transplantation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>LCH localized to the extrahepatic bile duct is extremely rare; however, LCH can still affect the extrahepatic bile ducts on occasion. LCH should be considered as a differential diagnosis if pediatric patients show the presence of sclerosing cholangitis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s40792-020-00899-6","type":"journal-article","created":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T09:03:45Z","timestamp":1592298225000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Langerhans cell histiocytosis confined to extrahepatic bile duct causing sclerosing cholangitis in child: a case report"],"prefix":"10.1186","volume":"6","author":[{"given":"Masakazu","family":"Murakami","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shun","family":"Onishi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuki","family":"Ohya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seiichi","family":"Kawabata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaori","family":"Isono","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhiko","family":"Sugawara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsuguharu","family":"Asato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yumi","family":"Honda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiki","family":"Mikami","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukihiro","family":"Inomata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taizo","family":"Hibi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1250-093X","authenticated-orcid":false,"given":"Satoshi","family":"Ieiri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,6,16]]},"reference":[{"key":"899_CR1","doi-asserted-by":"publisher","first-page":"1211","DOI":"10.5858\/arpa.2015-0199-RA","volume":"139","author":"CM Harmon","year":"2015","unstructured":"Harmon CM, Brown N. Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 2015;139:1211\u20134.","journal-title":"Arch Pathol Lab Med"},{"key":"899_CR2","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1016\/j.critrevonc.2004.01.002","volume":"50","author":"JI Henter","year":"2004","unstructured":"Henter JI, Tondini C, Pritchard J. Histiocyte disorders. Crit Rev Oncol Hematol. 2004;50:157\u201374.","journal-title":"Crit Rev Oncol Hematol"},{"key":"899_CR3","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1111\/ped.12380","volume":"56","author":"A Morimoto","year":"2014","unstructured":"Morimoto A, Oh Y, Shioda Y, Kudo K, Imamura T. Recent advances in Langerhans cell histiocytosis. Pediatr Int. 2014;56:451\u201361.","journal-title":"Pediatr Int"},{"key":"899_CR4","first-page":"437","volume":"35","author":"IE Obiorah","year":"2018","unstructured":"Obiorah IE, Velasquez AH, Kallakury B, \u00d6zdemirli M. Primary Langerhans cell histiocytosis of the extrahepatic bile duct occurring in an adult patient. Balkan Med J. 2018;35:437\u20139.","journal-title":"Balkan Med J"},{"key":"899_CR5","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1007\/s12185-013-1364-2","volume":"98","author":"K Horibe","year":"2013","unstructured":"Horibe K, Saito AM, Takimoto T, et al. Incidence and survival rates of hematological malignancies in Japanese children and adolescents (2006-2010): based on registry data from the Japanese Society of Pediatric Hematology. Int J Hematol. 2013;98:74\u201388.","journal-title":"Int J Hematol"},{"key":"899_CR6","first-page":"358","volume-title":"Tumours derived from Langerhans cells. WHO classification of tumours of haematopoietic and lymphoid tissues, 4th edn","author":"R Jaffe","year":"2008","unstructured":"Jaffe R, Weiss LM, Facchetti F. Tumours derived from Langerhans cells. WHO classification of tumours of haematopoietic and lymphoid tissues, 4th edn. Lyon: IARC; 2008. p. 358\u201360."},{"key":"899_CR7","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1002\/pbc.22224","volume":"54","author":"A Morimoto","year":"2010","unstructured":"Morimoto A, Ishida Y, Suzuki N, et al. Nationwide survey of single-system single site Langerhans cell histiocytosis in Japan. Pediatr Blood Cancer. 2010;54:98\u2013102.","journal-title":"Pediatr Blood Cancer"},{"key":"899_CR8","doi-asserted-by":"publisher","first-page":"461","DOI":"10.1080\/15513819709168587","volume":"17","author":"LS Finn","year":"1997","unstructured":"Finn LS, Jaffe R. Langerhans\u2019 cell granuloma confined to the bile duct. Pediatr Pathol Lab Med. 1997;17:461\u20138.","journal-title":"Pediatr Pathol Lab Med"},{"key":"899_CR9","doi-asserted-by":"publisher","first-page":"815","DOI":"10.1097\/00007890-200009150-00019","volume":"70","author":"N Hadzic","year":"2000","unstructured":"Hadzic N, Pritchard J, Webb D, et al. Recurrence of Langerhans cell histiocytosis in the graft after pediatric liver transplantation. Transplantation. 2000;70:815\u20139.","journal-title":"Transplantation."},{"key":"899_CR10","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1046\/j.1440-1746.1999.01919.x","volume":"14","author":"EA Roberts","year":"1999","unstructured":"Roberts EA. Primary sclerosing cholangitis in children. J Gastroenterol Hepatol. 1999;14:588\u201393.","journal-title":"J Gastroenterol Hepatol"},{"key":"899_CR11","doi-asserted-by":"publisher","first-page":"248","DOI":"10.12816\/0006047","volume":"7","author":"AA Al Salloom","year":"2013","unstructured":"Al Salloom AA, Almalki ST, Almana H, Burdelski M. Diabetes insipidus and sclerosing cholangitis in a child may be a clue to the diagnosis of Langerhans\u2019 cell histiocytosis: a case report. Int J Health Sci. 2013;7:248\u201351.","journal-title":"Int J Health Sci"}],"container-title":["Surgical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40792-020-00899-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40792-020-00899-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40792-020-00899-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,15]],"date-time":"2021-06-15T19:07:43Z","timestamp":1623784063000},"score":1,"resource":{"primary":{"URL":"https:\/\/surgicalcasereports.springeropen.com\/articles\/10.1186\/s40792-020-00899-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6,16]]},"references-count":11,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,12]]}},"alternative-id":["899"],"URL":"https:\/\/doi.org\/10.1186\/s40792-020-00899-6","relation":{},"ISSN":["2198-7793"],"issn-type":[{"value":"2198-7793","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,6,16]]},"assertion":[{"value":"3 April 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 June 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 June 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"This study was performed in accordance with the Ethical Guidelines for Medical and Health Research Involving Human Subjects by the Ministry of Health, Labour, and Welfare of Japan in 2014. The study complied with the 1964 Declaration of Helsinki (revised in 2013) and was approved by the local ethics committee of Kagoshima University Hospital (registration number: 27-119). Parents of the patient provided their informed consent for involvement in this study.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the parents of the patient for the publication of this case report and any accompanying images.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"137"}}