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Characteristics, management, and outcomes of patients with VIPoma-A retrospective analysis of the ENETS database
Philipps Univ, Dept Visceral Thorac & Vasc Surg, Marburg, Germany.;UKGM GmbH, Marburg, Germany..
Hop Edouard Herriot, Dept Med Oncol, Lyon, France..
Univ Paris Cite, Beaujon Hosp, Serv Pancreato Gastroenenterol, Clichy, France..
Gustave Roussy, Dept Imagerie Cancerol Endocrinienne, Villejuif, France..
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2026 (English)In: Journal of neuroendocrinology, ISSN 0953-8194, E-ISSN 1365-2826, Vol. 38, no 7, article id e70219Article in journal (Refereed) Published
Abstract [en]

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-world management, and outcomes of patients with VIPoma. Methods: Patients with VIPoma treated in a 20-year 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-free survival (DFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Results: Of 70 patients, 59 (54% male) with a median age of 55 years were included. Forty-six (78%) patients were diagnosed due to the classical watery diarrhea-hypokalemia-achlorhydria (WDHA) syndrome, and 40 (68%) presented with distant metastases at diagnosis. Fifty-two (88%) had serum VIP levels >2 times the upper normal limit and the median Ki-67 index was 5% (range 1%-40%). Surgery of the primary VIPoma was performed in 32 (54%), with curative intent in 22 (37%). In patients with stage I-III disease (n = 19), long-term cure was achieved in only 26% (5/19), with a median DFS of 81 (95%-CI: 1-215) months and 10-year OS of 78% (95%-CI: 61%-100%). In stage IV patients (n = 53, 40 at diagnosis, 13 disease progression), the sequence and type of 354 treatment lines varied, while SSA (n = 77, 22%), loco-regional liver-directed therapy (n = 72, 20%), and chemotherapy (n = 69, 19%) were most often applied. Median OS for patients with stage IV at diagnosis was 142 (95%-CI: 87-not available (NA)) months with 10-year survival of 50% (95%-CI: 35%-73%). Conclusion: Patients with VIPoma treated at NET centers have relatively favorable survival, even with distant metastases at diagnosis, although long-term cure rates remain low.

Place, publisher, year, edition, pages
John Wiley & Sons, 2026. Vol. 38, no 7, article id e70219
Keywords [en]
ENETS Center of Excellence, ENETS database, functional pancreatic neuroendocrine tumor, VIPoma
National Category
Cancer and Oncology Endocrinology and Diabetes
Identifiers
URN: urn:nbn:se:uu:diva-593854DOI: 10.1111/jne.70219ISI: 001800307800001PubMedID: 42338092Scopus ID: 2-s2.0-105042666822OAI: oai:DiVA.org:uu-593854DiVA, id: diva2:2085402
Available from: 2026-07-08 Created: 2026-07-08 Last updated: 2026-07-08Bibliographically approved

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Tiensuu Janson, Eva

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