{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T22:09:07Z","timestamp":1791410947503,"version":"4.3.3"},"reference-count":0,"publisher":"Oxford University Press (OUP)","issue":"4","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:sec>\n<jats:title><jats:italic>Purpose:<\/jats:italic><\/jats:title>\n<jats:p>We sought to refine the clinical picture of primary adrenal lymphoma (PAL), a rare lymphoid malignancy with predominant adrenal manifestation and risk of adrenal insufficiency.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title><jats:italic>Methods:<\/jats:italic><\/jats:title>\n<jats:p>Ninety-seven patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title><jats:italic>Results:<\/jats:italic><\/jats:title>\n<jats:p>Of the 81 patients with imaging data, 19 (23%) had isolated adrenal involvement (iPAL), while 62 (77%) had additional extra-adrenal involvement (PAL+). Among patients who had both CT and PET scans, 18FDG-PET revealed extra-adrenal involvement not detected by CT scan in 9\/18 cases (50%). The most common clinical manifestations were B symptoms (55%), fatigue (45%), and abdominal pain (35%). Endocrinological assessment was often inadequate. With a median follow-up of 41.6 months, 3-year progression-free (PFS) and overall (OS) survival rates in the entire cohort were 35.5% and 39.4%, respectively. The hazard ratios of iPAL for PFS and OS were 40.1 (95% CI: 2.63\u2013613.7, <jats:italic>P<\/jats:italic>\u2009=\u20090.008) and 2.69 (95% CI: 0.61\u201311.89, <jats:italic>P<\/jats:italic>\u2009=\u20090.191), respectively. PFS was much shorter in iPAL vs PAL+ (median 4 months vs not reached, <jats:italic>P<\/jats:italic>\u2009=\u20090.006), and OS also appeared to be shorter (median 16 months vs not reached), but the difference did not reach statistical significance (<jats:italic>P<\/jats:italic>\u2009=\u20090.16). Isolated PAL was more frequent in females (OR\u2009=\u20093.81; <jats:italic>P<\/jats:italic>\u2009=\u20090.01) and less frequently associated with B symptoms (OR\u2009=\u20090.159; <jats:italic>P<\/jats:italic>\u2009=\u20090.004).<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title><jats:italic>Conclusion:<\/jats:italic><\/jats:title>\n<jats:p>We found unexpected heterogeneity in the clinical spectrum of PAL. Further studies are needed to clarify whether clinical distinction between iPAL and PAL+ is corroborated by differences in molecular biology.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-19-0506","type":"journal-article","created":{"date-parts":[[2020,6,19]],"date-time":"2020-06-19T16:08:18Z","timestamp":1592582898000},"page":"453-462","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-7","source":"Crossref","is-referenced-by-count":30,"title":["Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study"],"prefix":"10.1093","volume":"183","author":[{"given":"Fatemeh","family":"Majidi","sequence":"first","affiliation":[{"name":"1Department of Hematology, Oncology and Clinical Immunology, Heinrich Heine University D\u00fcsseldorf, D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuela","family":"Martino","sequence":"additional","affiliation":[{"name":"1Department of Hematology, Oncology and Clinical Immunology, Heinrich Heine University D\u00fcsseldorf, D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mustafa","family":"Kondakci","sequence":"additional","affiliation":[{"name":"1Department of Hematology, Oncology and Clinical Immunology, Heinrich Heine University D\u00fcsseldorf, D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Antke","sequence":"additional","affiliation":[{"name":"19Klinik f\u00fcr Nuklearmedizin, Heinrich Heine Universit\u00e4t D\u00fcsseldorf, D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Haase","sequence":"additional","affiliation":[{"name":"2Department of Endocrinology, Heinrich Heine University D\u00fcsseldorf, D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vasileios","family":"Chortis","sequence":"additional","affiliation":[{"name":"3Center for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK"},{"name":"4Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wiebke","family":"Arlt","sequence":"additional","affiliation":[{"name":"3Center for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK"},{"name":"4Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina L","family":"Ronchi","sequence":"additional","affiliation":[{"name":"4Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK"},{"name":"5Divison of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Fassnacht","sequence":"additional","affiliation":[{"name":"5Divison of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"},{"name":"6Comprehensive Cancer Center Mainfranken, University of W\u00fcrzburg, W\u00fcrzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Laurent","sequence":"additional","affiliation":[{"name":"7University Hospital of Dijon, Dijon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Michel","family":"Petit","sequence":"additional","affiliation":[{"name":"7University Hospital of Dijon, Dijon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Casasnovas","sequence":"additional","affiliation":[{"name":"8Department of Hematology, University Hospital of Djion, Dijon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amir Mouhammed","family":"Habra","sequence":"additional","affiliation":[{"name":"9Department of Endocrine Neoplasia and Hormonal Disorders, MD Anderson Cancer Center, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleem","family":"Kanji","sequence":"additional","affiliation":[{"name":"10Baylor College of Medicine, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Salvatori","sequence":"additional","affiliation":[{"name":"11Division of Endocrinology, Diabetes and Metabolism, Johns Hopkins University, Baltimore, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"An Thi Nhat","family":"Ho","sequence":"additional","affiliation":[{"name":"20Department of Medicine, Medstar Harbor Hospital, Baltimore, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariadni","family":"Spyroglou","sequence":"additional","affiliation":[{"name":"12Klinik f\u00fcr Endokrinologie und Klinische Ern\u00e4hrung, Universit\u00e4tsspital Z\u00fcrich, Z\u00fcrich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Beuschlein","sequence":"additional","affiliation":[{"name":"12Klinik f\u00fcr Endokrinologie und Klinische Ern\u00e4hrung, Universit\u00e4tsspital Z\u00fcrich, Z\u00fcrich, Switzerland"},{"name":"13Klinik und Poliklinik IV, Klinikum der Universit\u00e4t M\u00fcnchen, Ludweig-Maximilians-Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diego","family":"Villa","sequence":"additional","affiliation":[{"name":"14BC Cancer Centre for Lymphoid Cancer and University of British Columbia, Vancouver, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wasithep","family":"Limvorapitak","sequence":"additional","affiliation":[{"name":"14BC Cancer Centre for Lymphoid Cancer and University of British Columbia, Vancouver, Canada"},{"name":"15Division of Hematology, Department of Internal Medicine, Thammasat University, Pathumthani, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bj\u00f6rn Engelbrekt","family":"Wahlin","sequence":"additional","affiliation":[{"name":"16Department of Medicine, Unit of Hematology, Karolinska Institute, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Gimm","sequence":"additional","affiliation":[{"name":"17Departments of Surgery, and Department of Biomedical and Clinical Sciences, Link\u00f6ping University, Link\u00f6ping, 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