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Deep phenotyping of patients along with evaluation of a transgenic murine model has led to the understanding of the role of HIF-2\u03b1 in developmental processes, including tumor development. Interestingly, pancreatic neuroendocrine tumors (NETs) occur in von Hippel-Lindau disease and the VHL gene product regulates HIF-2\u03b1 expression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Characterize pancreatic NETs in PZS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We have reviewed the clinical records in the index patient at Addenbrooke\u2019s Hospital (UK) and a cohort of patients with PZS from the NIH were assessed for pancreatic NETs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Herein, we describe a novel series from two institutions of patients with EPAS1-associated pancreatic neuroendocrine tumors including a case of a nonfunctioning pancreatic NET in association with an EPAS1 somatic mosaic variant.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case study extends our current understanding of the phenotypic spectrum in PZS and links pancreatic NETs to an additional hypoxia-associated gene, namely EPAS1.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/clinem\/dgag003","type":"journal-article","created":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T12:49:17Z","timestamp":1767962957000},"page":"1554-1563","source":"Crossref","is-referenced-by-count":1,"title":["Expanding the clinical tumor phenotype of the\n                    <i>EPAS1<\/i>\n                    -asssociated tumor syndrome"],"prefix":"10.1210","volume":"111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9492-5885","authenticated-orcid":false,"given":"Yasemin","family":"Cole","sequence":"first","affiliation":[{"name":"Department of Genomic Medicine, University of Cambridge , Cambridge CB2 0QQ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Howarth","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Cambridge University Hospital, Cambridge Biomedical Research Centre, Addenbrooke\u2019s Hospital , Cambridge CB2 0QQ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asna","family":"Javaid","sequence":"additional","affiliation":[{"name":"Molecular Genetic Laboratories, Cambridge University Hospital, Cambridge Biomedical Research Centre, Addenbrooke\u2019s Hospital , Cambridge CB2 0QQ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Tarpey","sequence":"additional","affiliation":[{"name":"Molecular Genetic Laboratories, Cambridge University Hospital, Cambridge Biomedical Research Centre, Addenbrooke\u2019s Hospital , Cambridge CB2 0QQ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Scoffings","sequence":"additional","affiliation":[{"name":"Department of Radiology, Cambridge University Hospital, Cambridge Biomedical Research Centre, Addenbrooke\u2019s Hospital , Cambridge CB2 0QQ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eamonn R","family":"Maher","sequence":"additional","affiliation":[{"name":"Department of Genomic Medicine, University of Cambridge , Cambridge CB2 0QQ ,","place":["UK"]},{"name":"Aston Medical School, College of Health and Life Sciences , Birmingham B4 7ET ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna L","family":"Godfrey","sequence":"additional","affiliation":[{"name":"Department of Haematology, Cambridge University Hospitals, NHS Foundation Trust , Cambridge CB2 0QQ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health (NIH) , Bethesda, MD 20892 ,","place":["USA"]},{"name":"Center for Adrenal Endocrine Tumors, AKESO , Prague 5 158 00 ,","place":["Czech Republic"]},{"name":"Multidisciplinary Consortium for Adrenal Diseases, Faculty of Medicine and Faculty Hospital, Palacky University , Olomouc 775 15 ,","place":["Czech Republic"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0009-7370","authenticated-orcid":false,"given":"Zhengping","family":"Zhuang","sequence":"additional","affiliation":[{"name":"Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health , Bethesda, MD 20892 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0861-9348","authenticated-orcid":false,"given":"Hussam","family":"Alkaissi","sequence":"additional","affiliation":[{"name":"National Institute of Diabetes and Digestive and Kidney Diseases, NIH , Bethesda, MD 20892 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4058-3135","authenticated-orcid":false,"given":"Ruth T","family":"Casey","sequence":"additional","affiliation":[{"name":"Department of Genomic Medicine, University of Cambridge , Cambridge CB2 0QQ 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