Pancreaticoduodenectomy Is the Best Surgical Procedure for Zollinger–Ellison Syndrome Associated with Multiple Endocrine Neoplasia Type 1

SIMPLE SUMMARY: Approximately 30% of patients with multiple endocrine neoplasia type 1 (MEN1) develop the Zollinger–Ellison syndrome (ZES), caused by solitary or multiple duodenal gastrinomas. Its management, especially regarding indication, timing, and type of surgery, is highly controversial. Therefore, the present study evaluated the long-term outcomes of pancreaticoduodenectomy (PD) versus non-PD resections in MEN1-ZES with regard to biochemical cure and quality of life. We found in a series of 35 patients that initial PD is the superior surgical procedure for MEN1-ZES, leading to long-term cure in about 80% of patients, fewer duodenopancreatic reoperations and an acceptable quality of life. Based on the results of this study, MEN1-ZES should be considered a surgically curable disease. ABSTRACT: Aim: The aim of this research was to evaluate the long-term outcome of pancreaticoduodenectomy (PD) versus other duodenopancreatic resections (non-PD) for the surgical treatment of the Zollinger–Ellison syndrome (ZES) in patients with multiple endocrine neoplasia type 1 (MEN1). Methods: Prospectively recorded patients with biochemically confirmed MEN1-ZES who underwent duodenopancreatic surgery were retrospectively analyzed in terms of clinical characteristics, complications, cure rate, and long-term morbidity, including quality of life assessment (EORTC QLQ-C30). Results: 35 patients (16 female, 19 male) with MEN1-ZES due to duodenopancreatic gastrinomas with a median age of 42 (range 30–74) years were included. At the time of diagnosis, 28 (80%) gastrinomas were malignant, but distant metastases were only present in one (3%) patient. Eleven patients (31.4%) underwent pancreatoduodenectomy (PD) as the initial procedure, whereas 24 patients underwent non-PD resections involving duodenotomy with gastrinoma excision, enucleation of the pNEN from the head of the pancreas, and peripancreatic lymphadenectomy, either with or without distal pancreatectomy (i.e., either Thompson procedure, n = 12, or DUODX, n = 12). There was no significant difference in perioperative morbidity and mortality between the two groups (p ≥ 0.05). One (9%) patient of the PD group required reoperation for recurrent or metastatic ZES compared to eight (22.8%) patients of the non-PD resection groups. After a median follow-up time of 134 months (range 6–480) nine of 11 (82%) patients in the PD group, two of 12 (16%) patients in the Thompson procedure group, and three of 12 (25%) patients in the DUODX group had normal serum gastrin levels. In addition, the global health QoLScore was better in the PD group (76.9) compared to the Thompson procedure (57.4) and DUODX (59.5) groups. Conclusions: Initial PD seems to be the superior surgical procedure for MEN1-ZES, resulting in a long-term cure rate of about 80%, fewer duodenopancreatic reoperations, and an acceptable quality of life.