{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T12:06:09Z","timestamp":1775477169062,"version":"3.50.1"},"reference-count":41,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,1,21]],"date-time":"2026-01-21T00:00:00Z","timestamp":1768953600000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100023322","name":"Szent-Gy\u00f6rgyi Albert Orvostudom\u00e1nyi Kar, Szegedi Tudom\u00e1nyegyetem","doi-asserted-by":"publisher","award":["5S 340 A202"],"award-info":[{"award-number":["5S 340 A202"]}],"id":[{"id":"10.13039\/501100023322","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100015269","name":"Magyarorsz\u00e1g Korm\u00e1nya","doi-asserted-by":"publisher","award":["\u00daNKP\u201021\u20104\u2010SZTE\u2010131"],"award-info":[{"award-number":["\u00daNKP\u201021\u20104\u2010SZTE\u2010131"]}],"id":[{"id":"10.13039\/501100015269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100015269","name":"Magyarorsz\u00e1g Korm\u00e1nya","doi-asserted-by":"publisher","award":["\u00daNKP\u201022\u20104\u2010305"],"award-info":[{"award-number":["\u00daNKP\u201022\u20104\u2010305"]}],"id":[{"id":"10.13039\/501100015269","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["APMIS"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Hemangioblastoma (HB) is a benign central nervous system (CNS) tumor associated with mutations in the von Hippel\u2013Lindau (\n                    <jats:italic>VHL<\/jats:italic>\n                    ) gene. Although rare outside the CNS, the pathological and genetic features remain poorly understood. We analyzed four renal hemangioblastomas (RHB). Demographics, clinical presentation, and follow\u2010up data were collected. After assessing hematoxylin and eosin\u2010stained slides, immunophenotyping was conducted using CA9, \u03b1\u2010inhibin, AE1\/AE3, CD10, CD56, PAX8, S100, MelanA, HMB45, CD117, FH, SDHB, and brachyury antibodies, alongside mismatch repair (MMR) deficiency examination. Additionally, whole\u2010exome sequencing (WES) was performed in 3 tumors. Our cohort comprised 3 male and 1 female patients, with a median age of 49\u2009years. No data on VHL disease were available. Well\u2010circumscribed tumors (median size: 25.5\u2009mm) displayed clear vacuolated cytoplasm with a vascular component. Immunostaining revealed expression of PAX8, \u03b1\u2010inhibin, AE1\/AE3, S100, and cytoplasmic brachyury. WES analysis detected no pathogenic mutations. No cancer\u2010related deaths or progressions were observed. Histologically, RHB resembles low\u2010grade ccRCC and shares expression of PAX8, pancytokeratin, and CA9. However, RHB is uniquely positive for \u03b1\u2010inhibin, S100, and lacks\n                    <jats:italic>VHL<\/jats:italic>\n                    alterations. Its favorable prognosis underscores the importance of distinguishing it from ccRCC to prevent unnecessary treatments. Further research is warranted to elucidate the underlying genetic mechanisms.\n                  <\/jats:p>","DOI":"10.1111\/apm.70147","type":"journal-article","created":{"date-parts":[[2026,1,21]],"date-time":"2026-01-21T08:37:34Z","timestamp":1768984654000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Hemangioblastoma of the Kidney\u2014A Comprehensive Clinical, Pathological, and Genetic Analysis of Four Cases"],"prefix":"10.1111","volume":"134","author":[{"given":"Bogl\u00e1rka","family":"P\u00f3sfai","sequence":"first","affiliation":[{"name":"Department of Pathology, Albert Szent\u2010Gy\u00f6rgyi Medical School University of Szeged  Szeged Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alex","family":"Jenei","sequence":"additional","affiliation":[{"name":"Department of Pathology and Experimental Cancer Research Semmelweis University  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gertr\u00fad","family":"Forika","sequence":"additional","affiliation":[{"name":"Department of Pathology and Experimental Cancer Research Semmelweis University  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Attila","family":"Fintha","sequence":"additional","affiliation":[{"name":"Department of Pathology and Experimental Cancer Research Semmelweis University  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zolt\u00e1n","family":"S\u00e1pi","sequence":"additional","affiliation":[{"name":"Department of Pathology and Experimental Cancer Research Semmelweis University  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c1ron","family":"Somor\u00e1cz","sequence":"additional","affiliation":[{"name":"Eurofins\u2010Medserv Ltd.  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Borb\u00e1la","family":"D\u00e9nes","sequence":"additional","affiliation":[{"name":"Eurofins\u2010Medserv Ltd.  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ferenc","family":"Salamon","sequence":"additional","affiliation":[{"name":"Pathology Unit Uzsoki Street Hospital  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Korn\u00e9lia Veronika","family":"Eizler","sequence":"additional","affiliation":[{"name":"Pathology Unit Fej\u00e9r County Szent Gy\u00f6rgy University Teaching Hospital  Sz\u00e9kesfeh\u00e9rv\u00e1r Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N\u00e1ndor","family":"Giba","sequence":"additional","affiliation":[{"name":"Pathology Unit Fej\u00e9r County Szent Gy\u00f6rgy University Teaching Hospital  Sz\u00e9kesfeh\u00e9rv\u00e1r Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D\u00e1vid","family":"Semj\u00e9n","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine and Clinical Center University of P\u00e9cs  P\u00e9cs Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ildik\u00f3","family":"Illy\u00e9s","sequence":"additional","affiliation":[{"name":"Department of Pathology, Insurance and Forensic Medicine Semmelweis University  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Krist\u00f3f Attila","family":"Kov\u00e1cs","sequence":"additional","affiliation":[{"name":"Department of Pathology, Insurance and Forensic Medicine Semmelweis University  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gy\u00f6ngyi","family":"Munk\u00e1csy","sequence":"additional","affiliation":[{"name":"Department of Bioinformatics Semmelweis University  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00e1nos","family":"Papp","sequence":"additional","affiliation":[{"name":"Department of Molecular Genetics and the National Tumor Biology Laboratory National Institute of Oncology  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fanni","family":"S\u00e1nta","sequence":"additional","affiliation":[{"name":"Department of Radiology, Brigham and Women's Hospital Harvard Medical School  Boston Massachusetts USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henriett","family":"Butz","sequence":"additional","affiliation":[{"name":"Department of Molecular Genetics and the National Tumor Biology Laboratory National Institute of Oncology  Budapest Hungary"},{"name":"HUN\u2010REN\u2010ONKOL\u2010TTK\u2010HCEMM Oncogenomics Research Group National Institute of Oncology  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9247-6679","authenticated-orcid":false,"given":"Levente","family":"Kuthi","sequence":"additional","affiliation":[{"name":"Department of Pathology and Experimental Cancer Research Semmelweis University  Budapest Hungary"},{"name":"HUN\u2010REN\u2010ONKOL\u2010TTK\u2010HCEMM Oncogenomics Research Group National Institute of Oncology  Budapest Hungary"},{"name":"Department of Surgical and Molecular Pathology Tumor Pathology Center, National Institute of Oncology  Budapest Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,1,21]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.4132\/jptm.2022.04.13"},{"key":"e_1_2_10_3_1","first-page":"363","article-title":"\u00d6r\u00f6kletes Vesetumor\u2010Szindr\u00f3m\u00e1k [Hereditary Renal Tumor Syndromes.]","volume":"164","author":"S\u00e1nta F.","year":"2023","journal-title":"Orvosi Hetilap"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAP.0000000000000176"},{"key":"e_1_2_10_5_1","article-title":"Current Knowledge and Prospects for Renal Hemangioblastoma and Renal Cell Carcinoma With Hemangioblastoma\u2010Like Features","volume":"11","author":"Kojima F.","year":"2023","journal-title":"Biomedicine"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/1746-1596-7-49"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3180457bfc"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1177\/1066896911434548"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181f2d9b8"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/1746-1596-7-39"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2015-202900"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2013.05.007"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13000-022-01213-8"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/03000605211027774"},{"key":"e_1_2_10_15_1","first-page":"1953","article-title":"Sporadic Hemangioblastoma of the Kidney With PAX2 and Focal CD10 Expression: Report of a Case","volume":"6","author":"Jiang J. 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