Article Dans Une Revue World Journal of Surgery Année : 2026

Surgical Management of Zollinger‐Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 an AFCE and GTE Cohort Study. (Association Francophone de Chirurgie Endocrinienne and Groupe d'étude des Tumeurs Endocrines)

1 CHU Pitié-Salpêtrière [AP-HP]
2 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
3 Hôpital Robert Debré
4 URCA - Université de Reims Champagne-Ardenne
5 Hôpital Edouard Herriot [CHU - HCL]
6 UCBL - Université Claude Bernard Lyon 1
7 Hôpital de Hautepierre [Strasbourg]
8 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
9 CHRU Brest - Centre Hospitalier Régional Universitaire de Brest
10 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
11 CHUGA - CHU de Grenoble-Alpes - Centre Hospitalier Universitaire CHU Grenoble
12 CHU Reims - Hôpital universitaire Robert Debré [Reims]
13 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
14 Hôpital Cochin [AP-HP]
15 U1018 (Équipe 2) - CESP - Oncostat
16 IGR - Institut Gustave Roussy
17 Hôpital Haut-Lévêque [CHU Bordeaux]
18 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
19 RESHAPE - Inserm U1290 - UCBL1 - Research on Healthcare Performance
20 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
21 CHU Caen Normandie - CHU Caen Normandie – Centre Hospitalier Universitaire de Caen Normandie
22 CHU-Liège - Centre Hospitalier Universitaire de Liège
23 ULiège - Université de Liège = University of Liège = Universiteit van Luik = Universität Lüttich
24 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
25 CHU Dijon - Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand
26 Hôpital Beaujon [AP-HP]
27 UPCité - Université Paris Cité
28 LA CONCEPTION - Hôpital de la Conception [CHU - APHM]
29 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
30 CIC - Centre d'Investigation Clinique [Rennes]
31 CHRO - Centre Hospitalier Régional d'Orléans
32 Hôpital Charles Nicolle [Rouen]
33 CIC-EC - Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques
34 EPICAD - Epidémiologie et recherche clinique en oncologie digestive : EPICAD (CTM UMR 1231)
Sébastien Gaujoux

Résumé

ABSTRACT Objective To describe surgical indications, procedures and outcomes in patients operated for Zollinger‐Ellison syndrome (ZES) in multiple endocrine neoplasia type 1 (MEN1) using a large nationwide cohort. Background Management of ZES in MEN1 remains controversial. Methods All patients with ZES diagnosed through the MEN1 AFCE/GTE network from 1985 to 2015. Results Among 233 ZES patients, 66 (28%) were operated for ZES‐related gastrinomas. Thirty‐three (51%) procedures aimed to remove gastrinomas and associated pancreatic neuroendocrine tumors (pNET(s)) with appropriate resection. Thirty‐two procedures (49%) aimed to remove gastrinomas alone (ZES group). Survival was decreased in patients metastatic at ZES diagnosis ( p < 0.001). Fifteen‐year survival among non‐metastatic patients was not significantly better in operated patients (82% vs. 70%, p = 0.2). Perioperative mortality was nil. Metastatic lymph nodes were found in 30/42 lymphadenectomies (71%). The choice between pancreaticoduodenectomy versus duodenal focused surgery in the ZES group was associated with pre‐operative detection of adenopathies ( p > 0.001), leading to more frequent lymphadenectomies ( p < 0.01). Previous pancreatic surgeries (30%) may have influenced the choice of ZES procedures. Gastrin levels were more frequently normalized when the duodenum and the head of pancreas were removed versus more localized duodenal surgeries ( p < 0.01). Conclusion The high rate of invaded nodes in lymphadenectomies in MEN1 patients operated for ZES, the absence of operative mortality, and the decreased survival in metastatic patients are indirect arguments for surgery. Pancreaticoduodenectomy may be indicated in young and fit individuals to better control hypergastrinemia and to prevent metastatic progression in the ZES group. Gastrinoma removal is justified when associated with large pNETs.

Management of ZES in MEN1 remains controversial.

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Dates et versions

hal-05562450 , version 2 (04-05-2026)
hal-05562450 , version 1 (05-05-2026)

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Sébastien Gaujoux, François Pattou, Guillaume Cadiot, Mustapha Adham, Philippe Bachellier, et al.. Surgical Management of Zollinger‐Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 an AFCE and GTE Cohort Study. (Association Francophone de Chirurgie Endocrinienne and Groupe d'étude des Tumeurs Endocrines). World Journal of Surgery, 2026, Online ahead of print. ⟨10.1002/wjs.70303⟩. ⟨hal-05562450v1⟩
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