{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T06:29:25Z","timestamp":1770272965518,"version":"3.49.0"},"reference-count":14,"publisher":"BMJ","issue":"4","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2021,3,11]]},"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:p>A 53-year-old man presented with a history of progressive abdominal distention for 1\u2009year. Physical examination revealed large palpable masses in the bilateral flank regions. Contrast-enhanced CT of the abdomen showed bilateral, symmetrical large perinephric masses with fat attenuating areas, which was further confirmed on MRI. CT of the paranasal sinuses revealed circumscribed extraconal soft tissue mass in the left orbit, causing scalloping and erosion of the left orbital roof. Fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography\/CT showed FDG uptake in the bilateral perinephric masses. Based on imaging appearance, a diagnosis of Erdheim-Chester disease (ECD) was suggested. Ultrasound-guided biopsy from perinephric masses revealed a sheet of histiocytes with sprinkled lymphocytes and plasma cells in the background. The histiocytes were immunopositive for CD68, S100 and immunonegative for CD1a, which confirmed the diagnosis of ECD. The patient was started on interferon-\u03b1-2a and showed symptomatic improvement.<\/jats:p>","DOI":"10.1136\/bcr-2020-239137","type":"journal-article","created":{"date-parts":[[2021,4,7]],"date-time":"2021-04-07T13:25:48Z","timestamp":1617801948000},"page":"e239137","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Erdheim-Chester disease: an unusual aetiology of bilateral lipomatous perinephric masses"],"prefix":"10.1136","volume":"14","author":[{"given":"Rashmi","family":"Singh","sequence":"first","affiliation":[{"name":"Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Priyanka","family":"Naranje","sequence":"additional","affiliation":[{"name":"Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Prashant","family":"Ramateke","sequence":"additional","affiliation":[{"name":"Pathology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nishikant Avinash","family":"Damle","sequence":"additional","affiliation":[{"name":"Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,4,7]]},"reference":[{"key":"2025082707103618000_14.4.e239137.1","doi-asserted-by":"crossref","first-page":"143","DOI":"10.3348\/jksr.2015.72.2.143","article-title":"Erdheim-Chester disease with perirenal masses containing macroscopic fat tissue","volume":"72","author":"Byeon","year":"2015","journal-title":"Journal of the Korean Society of Radiology"},{"key":"2025082707103618000_14.4.e239137.2","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfm121"},{"key":"2025082707103618000_14.4.e239137.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.crad.2016.05.014"},{"key":"2025082707103618000_14.4.e239137.4","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-01-643569"},{"key":"2025082707103618000_14.4.e239137.5","doi-asserted-by":"publisher","DOI":"10.1002\/art.22280"},{"key":"2025082707103618000_14.4.e239137.6","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-05-430140"},{"key":"2025082707103618000_14.4.e239137.7","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-03-561381"},{"key":"2025082707103618000_14.4.e239137.8","doi-asserted-by":"publisher","DOI":"10.1007\/s11926-014-0412-0"},{"key":"2025082707103618000_14.4.e239137.9","doi-asserted-by":"publisher","DOI":"10.12659\/AJCR.892750"},{"key":"2025082707103618000_14.4.e239137.10","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-137"},{"key":"2025082707103618000_14.4.e239137.11","doi-asserted-by":"crossref","DOI":"10.1016\/j.urology.2004.10.051","article-title":"Compression of kidneys in Erdheim-Chester disease of retroperitoneum: open surgical approach","volume":"65","author":"Wimpissinger","year":"2005","journal-title":"Urology"},{"key":"2025082707103618000_14.4.e239137.12","doi-asserted-by":"crossref","first-page":"54","DOI":"10.5114\/pjr.2018.73290","article-title":"Imaging findings in Erdheim-Chester disease: what every radiologist needs to know","volume":"83","author":"Kumar","year":"2018","journal-title":"Pol J Radiol"},{"key":"2025082707103618000_14.4.e239137.13","doi-asserted-by":"crossref","first-page":"1929","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":"2025082707103618000_14.4.e239137.14","doi-asserted-by":"crossref","first-page":"E114","DOI":"10.1002\/ajh.25055","article-title":"Phenotypes and survival in Erdheim-Chester disease: results from a 165-patient cohort","volume":"93","author":"Cohen-Aubart","year":"2018","journal-title":"Am J Hematol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2020-239137","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T14:10:42Z","timestamp":1756303842000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2020-239137"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,4]]},"references-count":14,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2021,4,7]]},"published-print":{"date-parts":[[2021,4]]}},"alternative-id":["10.1136\/bcr-2020-239137"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2020-239137","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,4]]}}}