{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T03:20:29Z","timestamp":1791602429540,"version":"4.3.4"},"reference-count":38,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T00:00:00Z","timestamp":1782172800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T00:00:00Z","timestamp":1782172800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Neuroendocrinology"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Introduction: VIPoma is an extremely rare functioning pancreatic neuroendocrine tumor. Therefore, data regarding treatment and outcome are very limited. Aim (s): This multicenter study aimed to analyze clinical characteristics, real\u2010world management, and outcomes of patients with VIPoma. Methods: Patients with VIPoma treated in a 20\u2010year period at 14 referral centers for neuroendocrine tumors (NET) were collected in the ENETS Database. Clinical characteristics, therapeutic interventions, and outcomes were analyzed retrospectively. Disease\u2010free survival (DFS) and overall survival (OS) were estimated using the Kaplan\u2013Meier method. Results: Of 70 patients, 59 (54% male) with a median age of 55\u2009years were included. Forty\u2010six (78%) patients were diagnosed due to the classical watery diarrhea\u2013hypokalemia\u2013achlorhydria (WDHA) syndrome, and 40 (68%) presented with distant metastases at diagnosis. Fifty\u2010two (88%) had serum VIP levels &gt;2 times the upper normal limit and the median Ki\u201067 index was 5% (range 1%\u201340%). Surgery of the primary VIPoma was performed in 32 (54%), with curative intent in 22 (37%). In patients with stage I\u2013III disease (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200919), long\u2010term cure was achieved in only 26% (5\/19), with a median DFS of 81 (95%\u2010CI: 1\u2013215) months and 10\u2010year OS of 78% (95%\u2010CI: 61%\u2013100%). In stage IV patients (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200953, 40 at diagnosis, 13 disease progression), the sequence and type of 354 treatment lines varied, while SSA (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200977, 22%), loco\u2010regional liver\u2010directed therapy (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200972, 20%), and chemotherapy (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200969, 19%) were most often applied. Median OS for patients with stage IV at diagnosis was 142 (95%\u2010CI: 87\u2013not available (NA)) months with 10\u2010year survival of 50% (95%\u2010CI: 35%\u201373%). Conclusion: Patients with VIPoma treated at NET centers have relatively favorable survival, even with distant metastases at diagnosis, although long\u2010term cure rates remain low.\n                  <\/jats:p>","DOI":"10.1111\/jne.70219","type":"journal-article","created":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T05:42:09Z","timestamp":1782279729000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Characteristics, management, and outcomes of patients with\n                    <scp>VIPoma<\/scp>\n                    \u2013A retrospective analysis of the\n                    <scp>ENETS<\/scp>\n                    database"],"prefix":"10.1111","volume":"38","author":[{"given":"Detlef K.","family":"Bartsch","sequence":"first","affiliation":[{"name":"Department of Visceral\u2010, Thoracic\u2010 and Vascular Surgery Philipps University and UKGM GmbH  Marburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Mais","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology H\u00f4pital Edouard Herriot  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9715-9189","authenticated-orcid":false,"given":"L.","family":"de Mestier","sequence":"additional","affiliation":[{"name":"Service de Pancreato\u2010Gastroenenterologie Beaujon Hospital, Universit\u00e9 Paris\u2010Cit\u00e9  Clichy France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Baudin","sequence":"additional","affiliation":[{"name":"D\u00e9partement d'Imagerie, Canc\u00e9rologie Endocrinienne Gustave Roussy  Villejuif France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Kaltsas","sequence":"additional","affiliation":[{"name":"1st Department of Internal and Propaedeutic Medicine, EKPA LAIKO ENETS and EURACAN 4 Center of Excellence National and Kapodistrian University of Athens  Athens Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Hofland","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Section of Endocrinology ENETS Centers of Excellence Erasmus MC Cancer Institute  Rotterdam Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Garcia\u2010Carbonero","sequence":"additional","affiliation":[{"name":"Oncology Department Hospital Universitario 12 de Octubre, IIS Imas12, Facultad de Medicina, Universidad Complutense de Madrid (UCM)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Tiensuu Janson","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences, Endocrine Oncology, and Science for Life Laboratory Uppsala University  Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. G.","family":"McNamara","sequence":"additional","affiliation":[{"name":"Department of Oncology University of Manchester\/The Christie NHS Foundation Trust  Manchester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Andreassen","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Endocrinology Rigshospitalet, and ENETS Center of Excellence Rigshospitalet University Kopenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Grozinsky\u2010Glasberg","sequence":"additional","affiliation":[{"name":"Neuroendocrine Tumor Unit, Division of Medicine and Faculty of Medicine the Hebrew University, Hadassah Medical Center  Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3642-0798","authenticated-orcid":false,"given":"F.","family":"Spada","sequence":"additional","affiliation":[{"name":"Division of Gastrointestinal Medical Oncology and Neuroendocrine Tumors European Institute of Oncology (IEO), IRCCS  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9080-2370","authenticated-orcid":false,"given":"H.","family":"Leupe","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine University Hospitals Leuven  Leuven Belgium"},{"name":"Department of Imaging and Pathology University of Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Panzuto","sequence":"additional","affiliation":[{"name":"Department of Medical\u2010Surgical Sciences and Translational Medicine, Sapienza University of Rome, Digestive Disease Unit Sant'Andrea University Hospital  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Fleschen","sequence":"additional","affiliation":[{"name":"ENETS Office Eurropean Neuroendocrine Tumor Society e.V.  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Klinkhammer","sequence":"additional","affiliation":[{"name":"Institute for Medical Biometry and Statistics University of Marburg  Marburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,23]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(58)90075-5"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/jne.13318"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPA.0000000000001347"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pan.2021.08.001"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0399-8320(04)95184-6"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.282.6278.1767"},{"key":"e_1_2_11_8_1","volume-title":"Endotext [Internet]","author":"Vinik 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