{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T12:17:54Z","timestamp":1787833074085,"version":"build-2784847793"},"reference-count":26,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T00:00:00Z","timestamp":1769126400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Int J Surg Pathol"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:p>Clear cell adenocarcinoma of the urinary tract is a rare subtype of urethral\/bladder cancer that is classified as a M\u00fcllerian-type tumor and is typically associated with more aggressive behavior and worse prognosis. Individuals with underlying congenital defects involving the urinary system are known to be at an increased risk for bladder cancer in general; however, it is currently unknown whether patients with aberrant M\u00fcllerian and Wolffian duct regression and\/or persistence, in addition to urinary anomalies, are at an increased risk for developing M\u00fcllerian-associated urinary tumors. In this case report, we present an extremely rare and unusual diagnosis of clear cell adenocarcinoma of the urinary tract arising in a male individual with Opitz syndrome, a congenital malformation disorder affecting multiple organ systems, including urogenital development. This presentation represents the first report of a urothelial tumor of M\u00fcllerian type occurring in a male individual with a urogenital congenital disorder.<\/jats:p>","DOI":"10.1177\/10668969251412914","type":"journal-article","created":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T12:52:56Z","timestamp":1769172776000},"page":"1074-1079","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Clear Cell Adenocarcinoma of the Urinary Tract Arising in a Young Man With the Congenital Malformation Disorder Opitz Syndrome: A Case Report"],"prefix":"10.1177","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6696-7909","authenticated-orcid":false,"given":"Jessica F.","family":"Williams","sequence":"first","affiliation":[{"name":"Brigham and Women\u2019s Hospital, Harvard Medical School"},{"name":"Department of Pathology, University of Utah Spencer Fox Eccles School of Medicine, and ARUP Laboratories, Salt Lake City, UT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9084-9654","authenticated-orcid":false,"given":"Juan F.","family":"Santoscoy Gutierrez","sequence":"additional","affiliation":[{"name":"Brigham and Women's Hospital, Harvard Medical School"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 I.","family":"Nolazco","sequence":"additional","affiliation":[{"name":"Harvard Medical School"},{"name":"Servicio de Urolog\u00eda, Hospital Universitario Austral, Universidad Austral, Pilar, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graeme S.","family":"Steele","sequence":"additional","affiliation":[{"name":"Harvard Medical School"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristine S.","family":"Burk","sequence":"additional","affiliation":[{"name":"Brigham and Women's Hospital, Harvard Medical School"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8767-8564","authenticated-orcid":false,"given":"Michelle S.","family":"Hirsch","sequence":"additional","affiliation":[{"name":"Brigham and Women\u2019s Hospital, Harvard Medical School"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalie M.","family":"Rizzo","sequence":"additional","affiliation":[{"name":"Brigham and Women\u2019s Hospital, Harvard Medical School"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,1,23]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.3390\/jcm9010138"},{"key":"e_1_3_2_3_2","volume-title":"Urinary and male genital tumours [Internet]. WHO classification of Tumours Series, 5th ed.; vol. 8","author":"WHO Classification of Tumours Editorial Board","year":"2022","unstructured":"WHO Classification of Tumours Editorial Board. Urinary and male genital tumours [Internet]. WHO classification of Tumours Series, 5th ed.; vol. 8. International Agency for Research on Cancer; 2022."},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11255-020-02725-2"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-07-4147"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2005.09.013"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/MOU.0000000000000297"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpurol.2017.05.006"},{"key":"e_1_3_2_9_2","first-page":"102202","article-title":"Many faces of Wilms tumor: Recent advances and future directions","volume":"64","author":"Bhutani N","year":"2021","unstructured":"Bhutani N, Kajal P, Sharma U. Many faces of Wilms tumor: Recent advances and future directions. Ann Med Surg (Lond). 2021;64:102202.","journal-title":"Ann Med Surg (Lond)"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.20931"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrendo.2014.83"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320020205"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1295-459"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1197-285"},{"key":"e_1_3_2_15_2","volume-title":"Tumors of the kidney, bladder, and related urinary structures","author":"Eble JN","year":"2022","unstructured":"Eble JN, Argani P, Grignon DJ, Cheng L. Tumors of the kidney, bladder, and related urinary structures. ARP Press; 2022."},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.16.17114"},{"issue":"1","key":"e_1_3_2_17_2","first-page":"790235","article-title":"Clear cell adenocarcinoma of the urethra: Review of the literature","volume":"2015","author":"Venyo AK-G","year":"2015","unstructured":"Venyo AK-G. Clear cell adenocarcinoma of the urethra: Review of the literature. Int J Surg Oncol. 2015;2015(1):790235.","journal-title":"Int J Surg Oncol"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.16.16929"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200202000-00005"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0046-8177(98)90015-6"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001589"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2009.10.011"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.4143\/crt.2023.577"},{"issue":"1","key":"e_1_3_2_24_2","first-page":"51","article-title":"Clear cell adenocarcinoma of the urinary tract primary to the renal pelvis","volume":"49","author":"Indulkar S","year":"2024","unstructured":"Indulkar S, Ribeiro E, Osunkoya AO, et al.. Clear cell adenocarcinoma of the urinary tract primary to the renal pelvis. Am J Surg Pathol. 2024;49(1):51\u201361 .","journal-title":"Am J Surg Pathol"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-019-02634-5"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10147-005-0497-6"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3835(00)00605-4"}],"container-title":["International Journal of Surgical Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/10668969251412914","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/10668969251412914","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/10668969251412914","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T11:22:44Z","timestamp":1787829764000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/10668969251412914"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,23]]},"references-count":26,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,6]]}},"alternative-id":["10.1177\/10668969251412914"],"URL":"https:\/\/doi.org\/10.1177\/10668969251412914","relation":{"has-review":[{"id-type":"doi","id":"10.1177\/10668969251412914\/v1\/review1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/10668969251412914\/v2\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/10668969251412914\/v1\/decision1","asserted-by":"object"},{"id-type":"doi","id":"10.1177\/10668969251412914\/v2\/response1","asserted-by":"object"}]},"ISSN":["1066-8969","1940-2465"],"issn-type":[{"value":"1066-8969","type":"print"},{"value":"1940-2465","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,23]]}}}