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)

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.