{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T04:47:05Z","timestamp":1790657225167,"version":"4.1.0"},"reference-count":36,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T00:00:00Z","timestamp":1759363200000},"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. Endocrinol."],"abstract":"<jats:sec><jats:title>Purpose<\/jats:title><jats:p>Primary adrenal lymphoma (PAL) is a rare malignancy with limited data on its characteristics. It can pose diagnostic challenges in differentiating it from other adrenal masses, such as atypical adrenal adenoma (ADE), pheochromocytoma (PCC), and adrenocortical carcinoma (ACC). This study aimed to characterize patients with PAL and compare their clinical, hormonal, and radiological features with other primary adrenal lesions whose computed tomography characteristics do not match those of a typical adenoma.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective, single-center study included four patient cohorts: PAL (n=12), ADE (n=31), PCC (n=18), and ACC (n=19), treated at a tertiary care hospital between January 2013 and January 2024.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The histopathological type of all PAL cases was diffuse large B-cell lymphoma (DLBCL). The median age at diagnosis was 70.5 (51\u201376) years. The diagnosis was more prevalent in males (sex ratio 1.4). Bilateral adrenal involvement was significantly more frequent in PAL patients. Clinical symptoms included general health deterioration (100%), weight loss (75%), abdominal pain (58%) and fever (41.7%). Laboratory assessments showed a higher prevalence of anemia in PAL patients compared to ADE and PCC patients. PAL cases exhibited elevated lactate dehydrogenase (LDH) and \u03b22-microglobulin as well as lipid profile abnormalities. Radiologically, PAL lesions were predominantly homogeneous, with a median tumor size of 78.5 (20-100.5) mm. All lesions exhibited an attenuation value &amp;gt; 20 Hounsfield Unit (HU) and lacked calcifications. Malignant lymph node involvement was significantly more frequent in the PAL than other cohorts.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>PAL should be included in the differential diagnosis of adrenal masses, particularly in cases of bilateral involvement. This study offers insights into its clinical presentation and highlights distinguishing features compared to other primary adrenal malignancies.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fendo.2025.1639878","type":"journal-article","created":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T04:21:53Z","timestamp":1759378913000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Primary adrenal lymphoma and its mimics: clinico-radiological differential diagnosis"],"prefix":"10.3389","volume":"16","author":[{"given":"Przemys\u0142aw","family":"K\u0142osowski","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Brzezi\u0144ska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piotr","family":"Kmie\u0107","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominika","family":"Okroj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Zembrzuska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariusz","family":"Kujawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Babi\u0144ska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renata","family":"\u015awi\u0105tkowska-Stodulska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,2]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdg004","article-title":"Primary extranodal non-Hodgkin\u2019s lymphoma (NHL): the impact of alternative definitions tested in the Comprehensive Cancer Centre West population-based NHL registry","volume":"14","author":"Krol","year":"2003","journal-title":"Ann Oncol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1093\/qjmed\/hcw174","article-title":"Adrenal lymphoma: presentation, management and prognosis","volume":"110","author":"Laurent","year":"2017","journal-title":"QJM"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.5603\/Hem.a2020.0023","article-title":"Primary adrenal lymphomas as interdisciplinary endocrinological and hematological problem: practical guidance on diagnosis and treatment","volume":"11","author":"Szyde\u0142ko","year":"2020","journal-title":"Hematol Clin Pract"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2020.00595","article-title":"Clinical features of 50 patients with primary adrenal lymphoma","volume":"11","author":"Wang","year":"2020","journal-title":"Front Endocrinol (Lausanne)"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3332\/ecancer.2016.634","article-title":"Acute adrenal insufficiency secondary to bilateral adrenal B-cell lymphoma: a case report and review of the literature","volume":"10","author":"De Miguel S\u00e1nchez","year":"2016","journal-title":"Ecancermedicalscience"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.4103\/2230-8210.85604","article-title":"Origin of primary adrenal lymphoma and predisposing factors for primary adrenal insufficiency in primary adrenal lymphoma","volume":"15","author":"Reddy","year":"2011","journal-title":"Indian J Endocrinol Metab"},{"key":"B7","doi-asserted-by":"publisher","first-page":"bcr2016219136","DOI":"10.1136\/bcr-2016-219136","article-title":"Rare cause of adrenal insufficiency","volume":"2017","author":"Hii","year":"2017","journal-title":"BMJ Case Rep"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1507\/endocrj.K08E-035","article-title":"Bilateral primary adrenal non-Hodgkin\u2019s lymphoma and primary adrenocortical carcinoma\u2014review of the literature preoperative differentiation of adrenal tumors","volume":"55","author":"Ozimek","year":"2008","journal-title":"Endocr J"},{"key":"B9","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1210\/endrev\/bnaa008","article-title":"Adrenal incidentaloma","volume":"41","author":"Sherlock","year":"2020","journal-title":"Endocr Rev"},{"key":"B10","doi-asserted-by":"publisher","first-page":"G1","DOI":"10.1093\/ejendo\/lvad066","article-title":"European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European Network for the Study of Adrenal Tumors","volume":"189","author":"Fassnacht","year":"2023","journal-title":"Eur J Endocrinol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.31916","article-title":"The who, when, and why of primary adrenal Malignancies: Insights into the epidemiology of a rare clinical entity","volume":"125","author":"Chandrasekar","year":"2019","journal-title":"Cancer"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.3390\/jcm12113739","article-title":"Review of diagnostic modalities for adrenal incidentaloma","volume":"12","author":"Okroj","year":"2023","journal-title":"J Clin Med"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1259\/bjr.20210642","article-title":"Imaging features of atypical adrenocortical adenomas: a radiological-pathological correlation","volume":"95","author":"Gargan","year":"2022","journal-title":"Br J Radiol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2021.636658","article-title":"A novel diagnostic model for primary adrenal lymphoma","volume":"12","author":"Yu","year":"2021","journal-title":"Front Endocrinol (Lausanne)"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-019-03740-9","article-title":"Clinical characteristics and outcomes of primary adrenal diffuse large B cell lymphoma in a large contemporary cohort: a SEER-based analysis","volume":"98","author":"Li","year":"2019","journal-title":"Ann Hematol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.3816\/CLM.2003.n.024","article-title":"Primary adrenal lymphoma","volume":"4","author":"Grigg","year":"2003","journal-title":"Clin Lymphoma"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-19-0506","article-title":"Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study","volume":"183","author":"Majidi","year":"2020","journal-title":"Eur J Endocrinol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-013-1812-3","article-title":"Primary adrenal lymphoma: a systematic review","volume":"92","author":"Rashidi","year":"2013","journal-title":"Ann Hematol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-05-1545","article-title":"Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray","volume":"103","author":"Hans","year":"2004","journal-title":"Blood"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2022.778984","article-title":"Primary adrenal lymphoma: two case series from China","volume":"12","author":"Zeng","year":"2022","journal-title":"Front Endocrinol (Lausanne)"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.3390\/curroncol29100623","article-title":"18F]FDG PET\/CT and PET\/MR in patients with adrenal lymphoma: a systematic review of literature and a collection of cases","volume":"29","author":"Evangelista","year":"2022","journal-title":"Curr Oncol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/j.mri.2021.03.009","article-title":"Handcrafted MRI radiomics and machine learning: classification of indeterminate solid adrenal lesions","volume":"79","author":"Stanzione","year":"2021","journal-title":"Magn Reson Imaging"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.18.20267","article-title":"Can texture analysis be used to distinguish benign from Malignant adrenal nodules on unenhanced CT, contrast-enhanced CT, or in-phase and opposed-phase MRI","volume":"212","author":"Ho","year":"2019","journal-title":"AJR Am J Roentgenol"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1155\/2019\/2641627","article-title":"Prognostic value of functional parameters of 18F-FDG PET images in patients with primary renal\/adrenal lymphoma","volume":"2019","author":"Wang","year":"2019","journal-title":"Contrast Media Mol Imaging"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19870701)60:1<103::AID-CNCR2820600119>3.0.CO;2-Y","article-title":"Prospective evaluation of adrenal insufficiency in patients with adrenal metastasis","volume":"60","author":"Redman","year":"1987","journal-title":"Cancer"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19840801)54:3<552::AID-CNCR2820540328>3.0.CO;2-R","article-title":"Metastases to the adrenal glands and the development of Addison\u2019s disease","volume":"54","author":"Seidenwurm","year":"1984","journal-title":"Cancer"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.3390\/jcm12155032","article-title":"Primary adrenal lymphomas with Cushing\u2019s syndrome: two cases with evidence of endogenous cortisol production by the neoplastic lymphoid cells","volume":"12","author":"Papageorgiou","year":"2023","journal-title":"J Clin Med"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1210\/jcem.85.2.6372","article-title":"A survey on adrenal incidentaloma in Italy. Study Group on Adrenal Tumors of the Italian Society of Endocrinology","volume":"85","author":"Mantero","year":"2000","journal-title":"J Clin Endocrinol Metab"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2011-1565","article-title":"Urine steroid metabolomics as a biomarker tool for detecting Malignancy in adrenal tumors","volume":"96","author":"Arlt","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"B30","doi-asserted-by":"publisher","first-page":"e942659","DOI":"10.12659\/AJCR.942659","article-title":"Uncovering primary extranodal diffuse large B cell lymphoma in the adrenal and thyroid glands","volume":"25","author":"Li","year":"2024","journal-title":"Am J Case Rep"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1530\/endoabs.50.PL1","article-title":"Primary hyperparathyroidism: molecular genetic insights and clinical implications. Presented at Society for Endocrinology BES 2017, Harrogate, UK","volume":"50","author":"Arnold","year":"2017","journal-title":"Endocr Abstr"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.3390\/cancers13133369","article-title":"The incidence and treatment response of double expression of MYC and BCL2 in patients with diffuse large B-cell lymphoma: a systematic review and meta-analysis","volume":"13","author":"Hwang","year":"2021","journal-title":"Cancers (Basel)"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2009.87","article-title":"Most primary adrenal lymphomas are diffuse large B-cell lymphomas with non-germinal center B-cell phenotype, BCL6 gene rearrangement and poor prognosis","volume":"22","author":"Mozos","year":"2009","journal-title":"Mod Pathol"},{"key":"B34","doi-asserted-by":"publisher","first-page":"e23173","DOI":"10.1002\/jcla.23173","article-title":"Adrenal diffuse large B-cell lymphoma with high PD-L1 expression: two case reports and literature review","volume":"34","author":"Chen","year":"2020","journal-title":"J Clin Lab Anal"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001386","article-title":"Morphologic patterns and the correlation with MYD88 L265P, CD79B mutations in primary adrenal diffuse large B-cell lymphoma","volume":"44","author":"Chen","year":"2020","journal-title":"Am J Surg Pathol"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1186\/1756-8722-5-49","article-title":"Prognostic factors in primary diffuse large B-cell lymphoma of adrenal gland treated with rituximab-CHOP chemotherapy from the Consortium for Improving Survival of Lymphoma (CISL)","volume":"5","author":"Kim","year":"2012","journal-title":"J Hematol Oncol"}],"container-title":["Frontiers in Endocrinology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2025.1639878\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T04:21:54Z","timestamp":1759378914000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2025.1639878\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,2]]},"references-count":36,"alternative-id":["10.3389\/fendo.2025.1639878"],"URL":"https:\/\/doi.org\/10.3389\/fendo.2025.1639878","relation":{},"ISSN":["1664-2392"],"issn-type":[{"value":"1664-2392","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,2]]},"article-number":"1639878"}}