{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:33:56Z","timestamp":1787063636786,"version":"build-2736575974"},"reference-count":105,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T00:00:00Z","timestamp":1769040000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder that typically occurs in lymph node-rich regions. Castleman disease originating in solid organs outside the lymph nodes is extremely rare. Current knowledge is limited to isolated case reports, leaving a lack of systematic evidence to guide diagnosis, management, and prognostic assessment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a systematic review of PubMed-reported cases and series of histologically confirmed of primary extranodal UCD over the past 20\u202fyears, summarizing their epidemiological, clinical, pathological, therapeutic features and outcome data.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 99 cases from 83 publications were included. The median age at diagnosis was 44\u202fyears, with a male-to-female ratio of 0.77:1. The mean tumor size was 4.9\u202fcm. The most frequent sites were the pancreas (14.1%), adrenal glands (14.1%), skeletal muscle (14.1%), parotid glands (13.1%), and kidneys (9.1%). Hyaline vascular type predominated (80.8%). Surgery was the primary treatment, yielding a 4-year recurrence-free survival rate of 84.6% (95% CI, 0.512\u20130.959). Lymphoma transformation occurred in 2% of cases. Radiotherapy and glucocorticoids may be effective treatment options.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Primary extranodal UCD displays different anatomic origins but generally carries an excellent prognosis following complete surgical resection. Awareness of its potential for malignant transformation is essential for long-term management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fmed.2026.1749931","type":"journal-article","created":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T06:56:05Z","timestamp":1769064965000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Retrospective analysis of primary extranodal unicentric Castleman disease: a systematic review"],"prefix":"10.3389","volume":"13","author":[{"given":"Jianing","family":"Shen","sequence":"first","affiliation":[{"name":"School of Basic Medical Sciences, Chengdu University of Traditional Chinese Medicine","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongjun","family":"Zeng","sequence":"additional","affiliation":[{"name":"Department of Pathology, Chengdu Integrated TCM and Western Medicine Hospital","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan","family":"Liu","sequence":"additional","affiliation":[{"name":"School of Basic Medical Sciences, Chengdu University of Traditional Chinese Medicine","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nie","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Oncology, The Research Institute of Integrated TCM & Western Medicine of Chengdu University of Chinese Medicine, Chengdu Integrated TCM and Western Medicine Hospital","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,1,22]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"822","DOI":"10.1002\/1097-0142(195607\/08)9:4<>3.0.co;2-4","article-title":"Localized mediastinal lymphnode hyperplasia resembling thymoma","volume":"9","author":"Castleman","year":"1956","journal-title":"Cancer"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1097\/00000478-198304000-00001","article-title":"A systemic lymphoproliferative disorder with morphologic features of Castleman's disease. 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