{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T07:28:36Z","timestamp":1772350116623,"version":"3.50.1"},"reference-count":49,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2023,4,29]],"date-time":"2023-04-29T00:00:00Z","timestamp":1682726400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Fonds Wetenschappelijk Onderzoek Vlaanderen","award":["FWO-V 1831017 N"],"award-info":[{"award-number":["FWO-V 1831017 N"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Von Hippel-Lindau (VHL) disease is a hereditary tumor syndrome that targets a highly selective subset of organs causing specific types of tumors. The biological basis for this principle of organ selectivity and tumor specificity is not well understood. VHL-associated hemangioblastomas share similar molecular and morphological features with embryonic blood and vascular precursor cells. Therefore, we suggest that VHL hemangioblastomas are derived from developmentally arrested hemangioblastic lineage keeping their potential of further differentiation. Due to these common features, it is of major interest to investigate whether VHL-associated tumors other than hemangioblastoma also share these pathways and molecular features. The expression of hemangioblast proteins has not yet been assessed in other VHL-related tumors. To gain a better understanding of VHL tumorigenesis, the expression of hemangioblastic proteins in different VHL-associated tumors was investigated. The expression of embryonic hemangioblast proteins Brachyury and TAL1 (T-cell acute lymphocytic leukemia protein 1) was assessed by immunohistochemistry staining on 75 VHL-related tumors of 51 patients: 47 hemangioblastomas, 13 clear cell renal cell carcinomas, 8 pheochromocytomas, 5 pancreatic neuroendocrine tumors, and 2 extra-adrenal paragangliomas. Brachyury and TAL1 expression was, respectively, observed in 26% and 93% of cerebellar hemangioblastomas, 55% and 95% of spinal hemangioblastomas, 23% and 92% of clear cell renal cell carcinomas, 38% and 88% of pheochromocytomas, 60% and 100% of pancreatic neuroendocrine tumors, and 50% and 100% of paragangliomas. We concluded that the expression of hemangioblast proteins in different VHL-associated tumors indicates a common embryological origin of these lesions. This may also explain the specific topographic distribution of VHL-associated tumors.<\/jats:p>","DOI":"10.3390\/cancers15092551","type":"journal-article","created":{"date-parts":[[2023,5,3]],"date-time":"2023-05-03T05:55:09Z","timestamp":1683093309000},"page":"2551","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Expression of Hemangioblast Proteins in von Hippel-Lindau Disease Related Tumors"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3862-3102","authenticated-orcid":false,"given":"Evelynn","family":"Vergauwen","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Vrije Universiteit Brussel, Laarbeeklaan 101, 1090 Jette, Belgium"},{"name":"Department of Neurology, Universiteit Antwerpen, Universiteitsplein 1, 2610 Antwerpen, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8337-5382","authenticated-orcid":false,"given":"Ramses","family":"Forsyth","sequence":"additional","affiliation":[{"name":"Department of Pathology, Universitair Ziekenhuis Brussel, Laarbeeklaan 101, 1090 Jette, Belgium"},{"name":"Experimental Pathology Research Group, Vrije Universiteit Brussel, Laarbeeklaan 103, 1090 Jette, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander","family":"Vortmeyer","sequence":"additional","affiliation":[{"name":"Department of Pathology, Indiana University-Purdue University, 420 University Blvd, Indianapolis, IN 46202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2553-0086","authenticated-orcid":false,"given":"Sven","family":"Gl\u00e4sker","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Vrije Universiteit Brussel, Laarbeeklaan 101, 1090 Jette, Belgium"},{"name":"Neurosurgery Section, Gesundheitsverbund Landkreis Konstanz (GLKN), 78224 Singen am Hohentwiel, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,4,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1038\/332268a0","article-title":"Von Hippel-Lindau Disease Maps to the Region of Chromosome 3 Associated with Renal Cell Carcinoma","volume":"332","author":"Seizinger","year":"1988","journal-title":"Nature"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1126\/science.8493574","article-title":"Identification of the Von Hippel-Lindau Disease Tumor Suppressor Gene","volume":"260","author":"Latif","year":"1993","journal-title":"Science"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1056\/NEJM197909133011107","article-title":"Hereditary Renal-Cell Carcinoma Associated with a Chromosomal Translocation","volume":"301","author":"Cohen","year":"1979","journal-title":"N. 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