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Partial Pancreaticoduodenectomy Can Provide Cure for Duodenal Gastrinoma Associated With Multiple Endocrine Neoplasia Type 1

Lopez, Caroline L. MD*; Falconi, Massimo MD; Waldmann, Jens MD*; Boninsegna, Letizia MD†,‡; Fendrich, Volker MD*; Goretzki, Peter K. MD§; Langer, Peter MD*; Kann, Peter H. MD; Partelli, Stefano MD†,‡; Bartsch, Detlef K. MD*

doi: 10.1097/SLA.0b013e3182536339
Original Articles

Objective: To evaluate the outcome of pancreaticoduodenectomy (PD) versus non-PD resections for the treatment of gastrinoma in multiple endocrine neoplasia type 1.

Background: Gastrinoma in MEN1 is considered a rarely curable disease and its management is highly controversial both for timing and extent of surgery.

Methods: Clinical characteristics, complications and outcomes of 27 prospectively collected MEN1 patients with biochemically proven gastrinoma, who underwent surgery, were analyzed with special regard to the gastrinoma type and the initial operative procedure.

Results: Twenty-two (81%) patients with gastrinoma in MEN1 had duodenal gastrinomas and 5 patients (19%) had pancreatic gastrinomas. At the time of diagnosis, 21 (77%) gastrinomas were malignant (18 duodenal, 3 pancreatic), but distant metastases were only present in 4 (15%) patients. Patients with pancreatic gastrinomas underwent either distal pancreatic resections or gastrinoma enucleation with lymphadenectomy, 2 patients also had synchronous resections of liver metastases. One of these patients was biochemically cured after a median of 136 (77–312) months. Thirteen patients with duodenal gastrinomas underwent PD resections (group 1, partial PD [n = 11], total PD [n = 2]), whereas 9 patients had no-PD resections (group 2) as initial operative procedure. Perioperative morbidity and mortality, including postoperative diabetes, differed not significantly between groups (P > 0.5). All patients of group 1 and 5 of 9 (55%) patients of group 2 had a negative secretin test at hospital discharge. However, after a median follow-up of 136 (3–276) months, 12 (92%) patients of group 1 were still normogastrinemic compared to only 3 of 9 (33%) patients of group 2 (P = 0.023). Three (33%) patients of group 2 had to undergo up to 3 reoperations for recurrent or metastatic disease compared to none of group 1.

Conclusions: Duodenal gastrinoma in MEN1 should be considered a surgically curable disease. PD seems to be the adequate approach to this disease, providing a high cure rate and acceptable morbidity compared to non-PD resections.

Duodenal gastrinoma in MEN1 should be considered a surgically curable disease. Partial pancreaticoduodenectomy seems to be the adequate approach to this disease, providing a high cure rate and acceptable morbidity.

*Department of Surgery, Philipps-University, Marburg, Germany

Department of Surgery, University of Verona, Italy

Department of Surgery, “Sacro Cuore Don Calabria” Hospital, Negrar, Italy

§Department of Surgery, Lukas Hospital Neuss, Germany

Division of Endocrinology and Diabetology, Philipps University Marburg, Germany.

Reprints: Detlef K. Bartsch, Department of Surgery, Philipps University Marburg, Baldingerstraße, D-35043 Marburg, Germany. E-mail: bartsch@med.uni-marburg.de.

C.L.L. and M.F. contributed equally.

Disclosure: The authors declare no conflicts of interest.

© 2013 Lippincott Williams & Wilkins, Inc.