{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T10:47:02Z","timestamp":1785754022980,"version":"3.56.0"},"reference-count":76,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T00:00:00Z","timestamp":1785715200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Von Hippel\u2013Lindau (VHL) disease is a rare autosomal dominant disorder predisposing individuals to multisystem neoplasms. While pheochromocytoma is a well-recognized endocrine manifestation of VHL, pancreatic involvement leading to clinically significant endocrine dysfunction is less frequently emphasized. We report a case of VHL syndrome in which diabetic ketoacidosis (DKA) occurred in the context of underlying pancreatic pathology, highlighting a potential but underexplored metabolic complication in these patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case summary<\/jats:title>\n                    <jats:p>A 31-year-old man came to the emergency department with a three-year history of polydipsia, polyuria, and hyperglycemia, and was diagnosed with diabetic ketoacidosis (DKA). His history included resection of cerebellar hemangioblastoma in 2009 and again in 2017, as well as subtotal pancreatectomy (head and body) for pancreatic lesions in 2017. At that time, his blood glucose was normal. His mother and maternal aunt both had hemangioblastoma. During this admission, we found severely impaired islet function in the residual pancreatic tail. Autoimmune diabetes was ruled out by negative anti-GAD, anti-IA-2, and anti-ZnT8 antibodies. There was no sign of infection or steroid use. Genetic testing showed a heterozygous deletion in exon 3 of the VHL gene, confirming VHL syndrome. With no other triggers identified, we attributed the DKA to acute decompensation from markedly reduced beta-cell reserve, likely due to both prior pancreatic resection and chronic VHL-related pancreatic disease. However, without serial imaging or histopathology to confirm progressive changes in the remnant tail, we could not establish a definitive causal relationship.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights that DKA can occur as an acute metabolic decompensation in patients with VHL-related pancreatic disease, particularly in those with prior pancreatic resections and progressive beta-cell loss over time. Nevertheless, the exact pathophysiological link between VHL-associated pancreatic involvement and the onset of DKA remains speculative and likely multifactorial. Clinicians should maintain vigilance for endocrine pancreatic insufficiency in VHL patients who present with unexplained hyperglycemia, even in the absence of a prior diabetes diagnosis. Further studies and accumulated case evidence are needed to clarify the mechanisms and risk factors for DKA in this specific population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fendo.2026.1880422","type":"journal-article","created":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T10:15:41Z","timestamp":1785752141000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Diabetic ketoacidosis associated with pancreatic involvement in a patient with von Hippel-Lindau syndrome: a case report"],"prefix":"10.3389","volume":"17","author":[{"given":"Yina","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Endocrinology and Metabolism, The Fourth Affiliated Hospital of Soochow University, Suzhou Dushu Lake Hospital, Medical Center of Soochow University","place":["Suzhou, Jiangsu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Futian","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, Kunshan Hospital of Chinese Medicine","place":["Suzhou, Jiangsu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xueqin","family":"Cao","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, The Fourth Affiliated Hospital of Soochow University, Suzhou Dushu Lake Hospital, Medical Center of Soochow University","place":["Suzhou, Jiangsu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, The Fourth Affiliated Hospital of Soochow University, Suzhou Dushu Lake Hospital, Medical Center of Soochow University","place":["Suzhou, Jiangsu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuzhu","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, The Fourth Affiliated Hospital of Soochow University, Suzhou Dushu Lake Hospital, Medical Center of Soochow University","place":["Suzhou, Jiangsu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism, The Fourth Affiliated Hospital of Soochow University, Suzhou Dushu Lake Hospital, Medical Center of Soochow University","place":["Suzhou, Jiangsu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,8,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-444-62702-5.00010-X","article-title":"Von Hippel-Lindau disease","volume":"132","author":"Chittiboina","year":"2015","journal-title":"Handb Clin Neurol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1186\/s12886-022-02661-y","article-title":"Von Hipple-Lindau disease complicated with central retinal vein occlusion: a case report","volume":"22","author":"Chen","year":"2022","journal-title":"BMC Ophthalmol"},{"key":"B3","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1186\/s13023-024-03060-w","article-title":"Epidemiology and economic burden of Von Hippel-Lindau Disease-associated central nervous system hemangioblastomas and pancreatic neuroendocrine tumors in the United States","volume":"19","author":"Jonasch","year":"2024","journal-title":"Orphanet J Rare Dis"},{"key":"B4","doi-asserted-by":"publisher","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":"B5","doi-asserted-by":"publisher","DOI":"10.2217\/fon-2023-0679","article-title":"Belzutifan: a novel therapeutic for the management of von Hippel-Lindau disease and beyond","volume":"20","author":"Curry","year":"2024","journal-title":"Future Oncol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1016\/s0161-6420(02)01177-6","article-title":"Treatment of retinal capillary hemangioma","volume":"109","author":"Singh","year":"2002","journal-title":"Ophthalmology"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(03)13643-4","article-title":"von Hippel-Lindau disease","volume":"361","author":"Lonser","year":"2003","journal-title":"Lancet"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1001\/jama.298.1.41","article-title":"Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel-Lindau disease","volume":"298","author":"Butman","year":"2007","journal-title":"JAMA"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2010.175","article-title":"von Hippel-Lindau disease: a clinical and scientific review","volume":"19","author":"Maher","year":"2011","journal-title":"Eur J Hum Genet"},{"key":"B10","first-page":"B4763","article-title":"Von Hippel-Lindau disease (vHL). National clinical guideline for diagnosis and surveillance in Denmark. 3rd edition","volume":"60","author":"Binderup","year":"2013","journal-title":"Dan Med J"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1001\/jama.277.18.1461","article-title":"Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease","volume":"277","author":"Manski","year":"1997","journal-title":"JAMA"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1053\/gast.2000.18143","article-title":"Pancreatic involvement in von Hippel-Lindau disease. The Groupe Francophone d'Etude de la Maladie de von Hippel-Lindau","volume":"119","author":"Hammel","year":"2000","journal-title":"Gastroenterology"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1002\/(sici)1098-1004(1998)12:6<417::aid-humu8>3.0.co;2-k","article-title":"Improved detection of germline mutations in the von Hippel-Lindau disease tumor suppressor gene","volume":"12","author":"Stolle","year":"1998","journal-title":"Hum Mutat"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33023","article-title":"Genotype-phenotype correlations in VHL exon deletions","author":"McNeill","year":"2009","journal-title":"Am J Med Genet A"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1016\/s1470-2045(25)00099-3","article-title":"Belzutifan for von Hippel-Lindau disease-associated renal cell carcinoma and other neoplasms (LITESPARK-004): 50 months follow-up from a single-arm, phase 2 study","volume":"26","author":"Srinivasan","year":"2025","journal-title":"Lancet Oncol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1056\/nejmoa2103425","article-title":"Belzutifan for Renal Cell Carcinoma in von Hippel-Lindau Disease","volume":"385","author":"Jonasch","year":"2021","journal-title":"N Engl J Med"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2025.43.5_suppl.534","article-title":"Belzutifan monotherapy in Chinese patients (pts) with von Hippel-Lindau (VHL) disease\u2013associated tumors: Results of LITESPARK-015 study","volume":"43","author":"Qiu","year":"2025","journal-title":"J Clin Oncol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/s2468-1253(16)30106-6","article-title":"Type 3c (pancreatogenic) diabetes mellitus secondary to chronic pancreatitis and pancreatic cancer","volume":"1","author":"Hart","year":"2016","journal-title":"Lancet Gastroenterol Hepatol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1097\/js9.0000000000000998","article-title":"Mapping global new-onset, worsening, and resolution of diabetes following partial pancreatectomy: a systematic review and meta-analysis","volume":"110","author":"Wei","year":"2024","journal-title":"Int J Surg"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.54781","article-title":"Association between pancreatic cysts and diabetes mellitus in von hippel-lindau disease","volume":"16","author":"Onishi","year":"2024","journal-title":"Cureus"},{"key":"B21","article-title":"Hepatic vascular tumors, angiectasis in multiple organs, and impaired spermatogenesis in mice with conditional inactivation of the VHL gene","volume":"63","author":"Ma","year":"2003","journal-title":"Cancer Res"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.3390\/cancers14041009","article-title":"Characterization of the pVHL Interactome in Human Testis Using High-Throughput Library Screening","volume":"14","author":"Falconieri","year":"2022","journal-title":"Cancers (Basel)"},{"key":"B23","doi-asserted-by":"publisher","first-page":"107417","DOI":"10.1016\/j.ijscr.2022.107417","article-title":"Von Hippel-Lindau disease: A case report","volume":"97","author":"Neupane","year":"2022","journal-title":"Int J Surg Case Rep"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1016\/j.amsu.2022.103292","article-title":"Retinal angioma of Von hippel-lindau disease: A case report","volume":"74","author":"Nabih","year":"2022","journal-title":"Ann Med Surg (Lond)"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.683021","article-title":"Central nervous system hemangioblastoma in a pediatric patient associated with von hippel-lindau disease: A case report and literature review","volume":"11","author":"Yang","year":"2021","journal-title":"Front Oncol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1002\/cai2.94","article-title":"Comprehensive treatment of von Hippel-Lindau disease: A case report","volume":"3","author":"Li","year":"2024","journal-title":"Cancer Innov"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2023.1296555","article-title":"Case report: Obstructive azoospermia as the first presentation of Von Hippel-Lindau disease","volume":"13","author":"Scafa","year":"2023","journal-title":"Front Oncol"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1016\/j.radcr.2023.09.009","article-title":"Von Hippel Lindau Disease with central nervous system and multiple visceral manifestations: A case report","volume":"18","author":"Tamang","year":"2023","journal-title":"Radiol Case Rep"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.20945\/2359-4292-2023-0181","article-title":"Expression of somatostatin receptors in hemangioblastomas associated with von Hippel-Lindau disease as a novel diagnostic, therapeutic, and follow-up opportunity: A case report and literature review","volume":"68","author":"Brabo","year":"2024","journal-title":"Arch Endocrinol Metab"},{"key":"B30","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1186\/s12872-022-02913-1","article-title":"Von Hippel-Lindau syndrome with a rare complication of dilated cardiomyopathy: a case report","volume":"22","author":"Yu","year":"2022","journal-title":"BMC Cardiovasc Disord"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.50484","article-title":"A case of von hippel-lindau disease with recurrence of paraganglioma and no other associated symptoms: the importance of genetic testing and establishing follow-up policies","volume":"15","author":"Okada","year":"2023","journal-title":"Cureus"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1016\/j.eucr.2021.101925","article-title":"A case of von Hippel-Lindau disease with renal cell carcinoma treated by partial nephrectomy with pre- and post-surgical axitinib therapy","volume":"40","author":"Akioka","year":"2022","journal-title":"Urol Case Rep"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.61702","article-title":"von hippel-lindau syndrome and secondary hypertension: A case report","volume":"16","author":"Bose","year":"2024","journal-title":"Cureus"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2022.963469","article-title":"Retinal hemangioblastoma in a patient with Von Hippel-Lindau disease: A case report and literature review","volume":"12","author":"Huang","year":"2022","journal-title":"Front Oncol"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1507\/endocrj.ej21-0611","article-title":"Retroperitoneal paraganglioma with loss of heterozygosity of the von Hippel-Lindau gene: a case report and review of the literature","volume":"69","author":"Anno","year":"2022","journal-title":"Endocr J"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijscr.2016.02.031","article-title":"Pancreatic manifestations in von Hippel-Lindau disease: A case report","volume":"21","author":"Ayloo","year":"2016","journal-title":"Int J Surg Case Rep"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.1177\/0300060518792368","article-title":"Effect of clarithromycin in von Hippel-Lindau syndrome: a case report","volume":"47","author":"Ma","year":"2019","journal-title":"J Int Med Res"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.12925","article-title":"Von hippel-lindau disease: A rare radiological case report of a symptomatic patient and his asymptomatic genetic counterpart","volume":"13","author":"Khanduri","year":"2021","journal-title":"Cureus"},{"key":"B39","article-title":"T-cell lymphoma with von Hippel-Lindau disease: a rare case report and review of literature","volume":"8","author":"Lou","year":"2015","journal-title":"Int J Clin Exp Pathol"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.4103\/jcrt.JCRT_127_18","article-title":"Adrenal adenoma in von Hippel-Lindau syndrome: A case report with review of literature","volume":"15","author":"Palui","year":"2019","journal-title":"J Cancer Res Ther"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijscr.2023.108481","article-title":"Total pancreatoduodenectomy for multiple pancreatic cysts in von Hippel-Lindau disease presenting as obstructive jaundice: A case report","volume":"109","author":"Jeo","year":"2023","journal-title":"Int J Surg Case Rep"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1155\/2020\/8824640","article-title":"A case of von hippel-lindau disease with bilateral pheochromocytoma and ectopic hypersecretion of intact parathyroid hormone in an adolescent girl","volume":"2020","author":"Belaid","year":"2020","journal-title":"Case Rep Endocrinol"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.3892\/ol.2014.2098","article-title":"Obstructive jaundice due to von Hippel-Lindau disease-associated pancreatic lesions: A case report","volume":"8","author":"Liang","year":"2014","journal-title":"Oncol Lett"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-010-0413-3","article-title":"A rare Von Hippel-Lindau disease that mimics acute myelitis: case report and review of the literature","volume":"32","author":"Jiang","year":"2011","journal-title":"Neurol Sci"},{"key":"B45","doi-asserted-by":"publisher","first-page":"358","DOI":"10.25259\/sni_304_2022","article-title":"Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel-Lindau disease: A case report and literature review","volume":"13","author":"Dantas","year":"2022","journal-title":"Surg Neurol Int"},{"key":"B46","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1186\/s12881-019-0930-8","article-title":"Novel gene mutation in von Hippel-Lindau disease - a report of two cases","volume":"20","author":"Wang","year":"2019","journal-title":"BMC Med Genet"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.12998\/wjcc.v9.i15.3711","article-title":"Anesthetic management of bilateral pheochromocytoma resection in Von Hippel-Lindau syndrome: A case report","volume":"9","author":"Wang","year":"2021","journal-title":"World J Clin cases"},{"key":"B48","doi-asserted-by":"publisher","first-page":"187","DOI":"10.25259\/sni_104_2024","article-title":"Cauda equina myxopapillary ependymoma in von Hippel-Lindau disease: A case report","volume":"15","author":"Ribeiro","year":"2024","journal-title":"Surg Neurol Int"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1016\/j.asjsur.2023.04.079","article-title":"Two tests of peripheral blood by standard methods were negative for Von Hippel-Lindau mutations: A case report","volume":"46","author":"Deng","year":"2023","journal-title":"Asian J Surg"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.1097\/md.0000000000005808","article-title":"Von Hippel-Lindau disease involving pancreas and biliary system: A rare case report","volume":"96","author":"Zhi","year":"2017","journal-title":"Med (Baltimore)"},{"key":"B51","doi-asserted-by":"publisher","first-page":"25","DOI":"10.6001\/actamedica.v27i1.4263","article-title":"Von Hippel-Lindau syndrome and renal tumours: radiological diagnostic and treatment options. A case report and literature review","volume":"27","author":"Untanas","year":"2020","journal-title":"Acta Med Litu"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.52760","article-title":"IL-6-producing pheochromocytoma associated with Von Hippel Lindau disease: a case report with literature review","volume":"16","author":"Hata","year":"2024","journal-title":"Cureus"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.5137\/1019-5149.jtn.6172-12.1","article-title":"Hemangioblastoma of the filum terminale associated with von Hippel-Lindau disease: a case report","volume":"23","author":"Tucer","year":"2013","journal-title":"Turk Neurosurg"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.1590\/0102-672020220002e1697","article-title":"Pancreatoduodenectomy in patient with Von Hippel-Lindau disease: a literature review","volume":"35","author":"Eulalio","year":"2022","journal-title":"Arq Bras Cir Dig"},{"key":"B55","doi-asserted-by":"publisher","DOI":"10.1016\/j.mjafi.2020.02.006","article-title":"Von Hippel-Lindau disease with extramedullary and pancreatic involvement","volume":"78","author":"Pantigozo-Rimachi","year":"2022","journal-title":"Med J Armed Forces India"},{"key":"B56","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijscr.2018.02.020","article-title":"Two-stage resection of a bilateral pheochromocytoma and pancreatic neuroendocrine tumor in a patient with von Hippel-Lindau disease: a case report","volume":"44","author":"Endo","year":"2018","journal-title":"Int J Surg Case Rep"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.3892\/ol.2015.3279","article-title":"Advanced renal cell carcinoma associated with von Hippel-Lindau disease: a case report and review of the literature","volume":"10","author":"Zhang","year":"2015","journal-title":"Oncol Lett"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.2176\/nmccrj.cr.2020-0143","article-title":"Metastasis of renal cell carcinoma to spinal hemangioblastoma in a patient with von Hippel-Lindau disease: a case report","volume":"8","author":"Wakita","year":"2021","journal-title":"NMC Case Rep J"},{"key":"B59","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1186\/s40792-017-0381-4","article-title":"Pancreatic neuroendocrine tumor with complete replacement of the pancreas by serous cystic neoplasms in a patient with von Hippel-Lindau disease: a case report","volume":"3","author":"Maeda","year":"2017","journal-title":"Surg Case Rep"},{"key":"B60","doi-asserted-by":"publisher","DOI":"10.12659\/ajcr.907356","article-title":"Von Hippel-Lindau disease with multi-organ involvement: a case report and 8-year clinical course with follow-up","volume":"18","author":"Yaghobi Joybari","year":"2017","journal-title":"Am J Case Rep"},{"key":"B61","doi-asserted-by":"publisher","DOI":"10.1007\/s12328-022-01684-8","article-title":"Tumor-to-tumor metastasis of colon cancer metastasizing to a pancreatic neuroendocrine tumor associated with von Hippel-Lindau disease: a case report","volume":"15","author":"Natsui","year":"2022","journal-title":"Clin J Gastroenterol"},{"key":"B62","doi-asserted-by":"publisher","DOI":"10.1007\/s13730-022-00770-7","article-title":"Robot-assisted sutureless partial nephrectomy for the treatment of fifteen bilateral renal masses in a patient with Von Hippel-Lindau syndrome: a case report from a high-volume robotic center","volume":"12","author":"Peraire Lores","year":"2023","journal-title":"CEN Case Rep"},{"key":"B63","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.8701-21","article-title":"The first case of gastric neuroendocrine tumors induced by a proton pump inhibitor in von Hippel-Lindau disease","volume":"61","author":"Kawaguchi","year":"2022","journal-title":"Intern Med"},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.3390\/ijms26062751","article-title":"Mosaic form of von Hippel-Lindau syndrome: case report and literature review","volume":"26","author":"Mikhaylenko","year":"2025","journal-title":"Int J Mol Sci"},{"key":"B65","doi-asserted-by":"publisher","DOI":"10.4103\/jcrt.jcrt_718_18","article-title":"Aggressive-fungating retinal hemangioblastoma","volume":"17","author":"Menon","year":"2021","journal-title":"J Cancer Res Ther"},{"key":"B66","doi-asserted-by":"publisher","DOI":"10.4143\/crt.2014.299","article-title":"A case of von Hippel-Lindau disease with colorectal adenocarcinoma, renal cell carcinoma and hemangioblastomas","volume":"48","author":"Heo","year":"2016","journal-title":"Cancer Res Treat"},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.3892\/ol.2016.4387","article-title":"Long-term follow-up and clinical course of a rare case of von Hippel-Lindau disease: a case report and review of the literature","volume":"11","author":"Zou","year":"2016","journal-title":"Oncol Lett"},{"key":"B68","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1186\/s12881-020-01126-7","article-title":"Intronic mutation of the VHL gene associated with central nervous system hemangioblastomas in two Chinese families with Von Hippel-Lindau disease: case report","volume":"21","author":"Liu","year":"2020","journal-title":"BMC Med Genet"},{"key":"B69","doi-asserted-by":"publisher","DOI":"10.1210\/jcemcr\/luae097","article-title":"An unusual case of pheochromocytoma associated with von Hippel-Lindau disease and Lynch syndrome during pregnancy","volume":"2","author":"Tang","year":"2024","journal-title":"JCEM Case Rep"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.1159\/000480372","article-title":"Acinar cell cystadenoma - a rarity in advanced von Hippel-Lindau disease: a case report","volume":"34","author":"Fahlbusch","year":"2018","journal-title":"Visc Med"},{"key":"B71","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijscr.2013.03.004","article-title":"Von Hippel-Lindau and myotonic dystrophy of Steinert along with pancreatic neuroendocrine tumor and renal clear cell carcinomal neoplasm: case report and review of the literature","volume":"4","author":"Addeo","year":"2013","journal-title":"Int J Surg Case Rep"},{"key":"B72","doi-asserted-by":"publisher","DOI":"10.1186\/1477-7819-12-74","article-title":"Superior mediastinal paraganglioma associated with von Hippel-Lindau syndrome: report of a case","volume":"12","author":"Takahashi","year":"2014","journal-title":"World J Surg Oncol"},{"key":"B73","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.49749","article-title":"Anesthetic challenge in a parturient with Von Hippel-Lindau disease","volume":"15","author":"Lye","year":"2023","journal-title":"Cureus"},{"key":"B74","doi-asserted-by":"publisher","DOI":"10.1093\/jscr\/rjae619","article-title":"Follow-up report on pulmonary mucosa-associated lymphoma in a patient with von Hippel-Lindau disease","volume":"2024","author":"Olarewaju","year":"2024","journal-title":"J Surg Case Rep"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.5489\/cuaj.2863","article-title":"Sorafenib treatment for recurrent stage T1 bilateral renal cell carcinoma in patients with Von Hippel-Lindau disease: a case report and literature review","volume":"9","author":"Choi","year":"2015","journal-title":"Can Urol Assoc J"},{"key":"B76","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijscr.2023.108188","article-title":"Von-Hippel Lindau (VHL) syndrome with bilateral cerebellar hemangioblastomas and retinal angiomas: a rare presentation","volume":"106","author":"Acharya","year":"2023","journal-title":"Int J Surg Case Rep"}],"container-title":["Frontiers in Endocrinology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2026.1880422\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T10:15:42Z","timestamp":1785752142000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2026.1880422\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,3]]},"references-count":76,"alternative-id":["10.3389\/fendo.2026.1880422"],"URL":"https:\/\/doi.org\/10.3389\/fendo.2026.1880422","relation":{},"ISSN":["1664-2392"],"issn-type":[{"value":"1664-2392","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,3]]},"article-number":"1880422"}}