{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T11:51:46Z","timestamp":1753876306713,"version":"3.41.2"},"reference-count":15,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2021,9,7]],"date-time":"2021-09-07T00:00:00Z","timestamp":1630972800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,10,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Erdheim\u2013Chester disease (ECD) is a rare non-Langerhans cell histiocytosis that can affect the bones, heart, lungs, brain, and other organs. Cardiovascular involvement is common in ECD and is associated with a poor prognosis. Here, we report a case of ECD presenting as an intracardiac mass and pericardial effusion confirmed by biopsy with sternotomy.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>A 54-year-old man was admitted because of dyspnoea. He was previously diagnosed with bilateral hydronephrosis and retroperitoneal fibrosis. Echocardiography revealed a large amount of pericardial effusion and echogenic mass on the right atrial (RA) side and atrioventricular (AV) groove. Cardiac magnetic resonance imaging and positron emission tomography-computed tomography (CT) revealed infiltrative mass-like lesions in the RA and AV groove. Pericardial window formation and pericardial biopsy were performed, and the pathologic results showed only pericardial fibrosis with no specific findings. Bone scan revealed increased uptake in the long bones. Considering the high probability of ECD based on the patient\u2019s manifestations and the imaging findings, we performed a cardiac biopsy with median sternotomy despite initial insufficient pathologic results in the pericardial biopsy. The surgical findings included multiple irregular and firm masses on the cardiac wall and large vessels; after obtaining a large amount of suspicious mass, ECD accompanied with CD68 (+) and BRAF V600E mutation was confirmed.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>Erdheim\u2013Chester disease can be associated with various forms of cardiovascular involvement. Considering the multi-systemic manifestations and difficulty in identifying this rare disease, a comprehensive and meticulous diagnostic work-up is crucial.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytab351","type":"journal-article","created":{"date-parts":[[2021,9,7]],"date-time":"2021-09-07T14:51:31Z","timestamp":1631026291000},"source":"Crossref","is-referenced-by-count":5,"title":["Erdheim\u2013Chester disease presenting as an intracardiac mass and pericardial effusion confirmed by biopsy: a case report"],"prefix":"10.1093","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4209-655X","authenticated-orcid":false,"given":"Minjae","family":"Yoon","sequence":"first","affiliation":[{"name":"Division of Cardiology, Department of Internal Medicine, Severance Cardiovascular Hospital, Yonsei University College of Medicine, 50-1, Yonsei-Ro, Seodaemun-gu, Seoul 03722, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0311-6565","authenticated-orcid":false,"given":"Seung Hyun","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5718-3624","authenticated-orcid":false,"given":"Hyo Sup","family":"Shim","sequence":"additional","affiliation":[{"name":"Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seok-Min","family":"Kang","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Internal Medicine, Severance Cardiovascular Hospital, Yonsei University College of Medicine, 50-1, Yonsei-Ro, Seodaemun-gu, Seoul 03722, Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2021,9,7]]},"reference":[{"key":"2021101504444925800_ytab351-B1","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1182\/blood-2014-03-561381","article-title":"Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease","volume":"124","author":"Diamond","year":"2014","journal-title":"Blood"},{"key":"2021101504444925800_ytab351-B2","doi-asserted-by":"crossref","first-page":"e1365","DOI":"10.1097\/MD.0000000000001365","article-title":"Cardiovascular involvement in Erdheim-Chester disease: a case report and review of the literature","volume":"94","author":"Nicolazzi","year":"2015","journal-title":"Medicine (Baltimore)"},{"key":"2021101504444925800_ytab351-B3","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1007\/BF01942684","article-title":"\u00dcber Lipoidgranulomatose","volume":"279","author":"Chester","year":"1930","journal-title":"Virchows Arch Path Anat"},{"key":"2021101504444925800_ytab351-B4","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1016\/j.ejim.2015.03.004","article-title":"Erdheim-Chester disease","volume":"26","author":"Campochiaro","year":"2015","journal-title":"Eur J Intern Med"},{"key":"2021101504444925800_ytab351-B5","first-page":"S","article-title":"Cardiovascular manifestations of Erdheim-Chester disease","volume":"33","author":"Berti","year":"2015","journal-title":"Clin Exp Rheumatol"},{"key":"2021101504444925800_ytab351-B6","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1007\/s11926-014-0412-0","article-title":"Erdheim\u2013Chester disease","volume":"16","author":"Haroche","year":"2014","journal-title":"Curr Rheumatol Rep"},{"key":"2021101504444925800_ytab351-B7","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1097\/01.md.0000145368.17934.91","article-title":"Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review","volume":"83","author":"Haroche","year":"2004","journal-title":"Medicine (Baltimore)"},{"key":"2021101504444925800_ytab351-B8","doi-asserted-by":"crossref","first-page":"e597","DOI":"10.1161\/CIRCULATIONAHA.108.825075","article-title":"Images in cardiovascular medicine. 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