{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T00:51:29Z","timestamp":1787014289306,"version":"build-2736575974"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,8,15]],"date-time":"2025-08-15T00:00:00Z","timestamp":1755216000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Renal haemangioblastoma is a very rare mesenchymal neoplasm that remains incompletely characterized despite its inclusion in the WHO classification. In this study, we investigate the clinicopathologic and molecular features of renal haemangioblastoma.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>\n                      The cohort comprised 8 patients (six male, two female) with a median age of 47\u2009years (range: 29\u201363). No patients had a known history of a tumour predisposition syndrome. The tumours ranged in size from 1.3 to 8.0\u2009cm (median: 4.0\u2009cm). Histologically, the tumours were well circumscribed and composed of epithelioid\/polygonal cells with clear\u2010to\u2010palely eosinophilic and often multivacuolated cytoplasm. In other areas, the tumour cells were more spindled. A prominent vascular pattern was frequently present. Two tumours were closely associated with renal cell carcinoma (RCC); the latter demonstrated features characteristic of RCC with fibromyomatous stroma (FMS). Targeted DNA sequencing was successfully performed on 7 tumours, revealing that most (5\/7; 71%) harboured one or more alterations involving mTOR pathway genes. A total of 9 mTOR pathway alterations were identified, affecting\n                      <jats:italic>MTOR<\/jats:italic>\n                      (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093),\n                      <jats:italic>TSC1<\/jats:italic>\n                      (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094),\n                      <jats:italic>TSC2<\/jats:italic>\n                      (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091) and\n                      <jats:italic>PTEN<\/jats:italic>\n                      (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091). Immunohistochemistry for glycoprotein nonmetastatic B (GPNMB) was positive in 5\/6 tumours assessed, including one tumour that lacked an identifiable mTOR pathway alteration by sequencing.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This study contributes further understanding to the pathogenesis of renal haemangioblastoma and supports that it exists on a spectrum with RCC with haemangioblastoma\u2010like features and RCC with FMS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/his.15538","type":"journal-article","created":{"date-parts":[[2025,8,15]],"date-time":"2025-08-15T13:02:08Z","timestamp":1755262928000},"page":"726-735","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Renal haemangioblastoma: a clinicopathologic and molecular characterization of 8 cases supporting the presence of recurrent\n                    <scp>MTOR<\/scp>\n                    pathway alterations"],"prefix":"10.1111","volume":"87","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3439-7013","authenticated-orcid":false,"given":"Maria Del Carmen","family":"Rodriguez Pena","sequence":"first","affiliation":[{"name":"Department of Pathology Brigham and Women's Hospital and Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shujin","family":"He","sequence":"additional","affiliation":[{"name":"Department of Pathology, The First Affiliated Hospital Sun Yat\u2010Sen University  Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1848-2389","authenticated-orcid":false,"given":"Stephanie","family":"Siegmund","sequence":"additional","affiliation":[{"name":"Department of Pathology Brigham and Women's Hospital and Harvard Medical School  Boston Massachusetts USA"}],"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":"Department of Pathology Brigham and Women's Hospital and Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3357-5348","authenticated-orcid":false,"given":"Anjia","family":"Han","sequence":"additional","affiliation":[{"name":"Department of Pathology, The First Affiliated Hospital Sun Yat\u2010Sen University  Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5807-7831","authenticated-orcid":false,"given":"William J","family":"Anderson","sequence":"additional","affiliation":[{"name":"Department of Pathology Brigham and Women's Hospital and Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,8,15]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2613.2007.00535.x"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182a266c1"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3180457bfc"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2015-202900"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAP.0000000000000176"},{"key":"e_1_2_10_7_1","volume-title":"WHO classification of Tumours urinary and male genital Tumours","author":"Board 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knowledge and prospects for renal hemangioblastoma and renal cell carcinoma with hemangioblastoma\u2010like features","volume":"11","author":"Kojima 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