{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T05:04:56Z","timestamp":1780031096354,"version":"3.53.1"},"reference-count":11,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T00:00:00Z","timestamp":1780012800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Erdheim\u2013Chester disease (ECD) is a rare form of histiocytosis with various clinical manifestations. Liver involvement has been infrequently reported.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>We report a case of ECD with liver involvement, presenting with progressive elevation of liver enzymes, a manifestation rarely reported in the literature. Follow-up liver function testing and FAPI-PET\/CT performed 6 months after initiation of trametinib therapy demonstrated a dramatic response.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Trametinib holds promise as a therapeutic option for patients with non-BRAF-V600E mutant ECD presenting with liver involvement.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fonc.2026.1740236","type":"journal-article","created":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T04:46:29Z","timestamp":1780029989000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Erdheim\u2013Chester disease with liver involvement successfully treated with trametinib"],"prefix":"10.3389","volume":"16","author":[{"given":"Long","family":"Chang","sequence":"first","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College","place":["Beijing, China"]},{"name":"State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai-ni","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College","place":["Beijing, China"]},{"name":"State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ya-Ping","family":"Luo","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital","place":["Beijing, China"]},{"name":"Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xin-xin","family":"Cao","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center, Chinese Academy of Medical Sciences and Peking Union Medical College","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,29]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2012-eular.1506","article-title":"Erdheim-chester disease","volume":"135","author":"Haroche","year":"2020","journal-title":"Blood"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-021-01439-3","article-title":"Diverse kinase alterations and myeloid-associated mutations in adult histiocytosis","volume":"36","author":"Chen","year":"2022","journal-title":"Leukemia"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019003507","article-title":"Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era","volume":"135","author":"Goyal","year":"2020","journal-title":"Blood"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.14309\/crj.2017.95","article-title":"A fatal case of Erdheim-Chester disease with hepatic involvement","volume":"4","author":"Balasubramanian","year":"2017","journal-title":"ACG Case Rep J"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1572-0241.2006.00893.x","article-title":"Biliary manifestation of Erdheim-Chester disease mimicking Klatskin's carcinoma","volume":"102","author":"Gundling","year":"2007","journal-title":"Am J Gastroenterol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"280","DOI":"10.1111\/j.1365-2141.2007.06642.x","article-title":"Multisystem Erdheim-Chester disease; a unique presentation with liver and axial skeletal involvement","volume":"138","author":"Gupta","year":"2007","journal-title":"Br J Haematol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.5858\/2003-127-e337-edaupw","article-title":"Erdheim-Chester disease: a unique presentation with liver involvement and vertebral osteolytic lesions","volume":"127","author":"Ivan","year":"2003","journal-title":"Arch Pathol Lab Med"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1186\/1471-230x-11-77","article-title":"Unusual manifestation of Erdheim-Chester disease","volume":"11","author":"Pan","year":"2011","journal-title":"BMC Gastroenterol"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.4132\/jptm.2017.10.16","article-title":"Erdheim-Chester disease involving lymph nodes and liver clinically mimicking lymphoma: a case report","volume":"52","author":"Sung","year":"2018","journal-title":"J Pathol Transl Med"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1093\/oncolo\/oyae175","article-title":"Liver involvement with Langerhans cell histiocytosis in adults","volume":"29","author":"Chang","year":"2024","journal-title":"Oncologist"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.2967\/jnumed.123.265691","article-title":"Performance of (68)Ga-labeled fibroblast activation protein inhibitor PET\/CT in evaluation of Erdheim-Chester disease: a comparison with (18)F-FDG PET\/CT","volume":"64","author":"Ma","year":"2023","journal-title":"J Nucl Med"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1740236\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T04:46:54Z","timestamp":1780030014000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1740236\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,29]]},"references-count":11,"alternative-id":["10.3389\/fonc.2026.1740236"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1740236","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,29]]},"article-number":"1740236"}}