title: Partial pancreaticoduodenectomy can provide cure for duodenal gastrinoma associated with multiple endocrine neoplasia type 1.
abstract: 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.
authors.affiliation: Department of Surgery, Philipps-University, Marburg, Germany.
authors.forename: Caroline L
authors.initials: CL
authors.lastname: Lopez
authors.forename: Massimo
authors.initials: M
authors.lastname: Falconi
authors.forename: Jens
authors.initials: J
authors.lastname: Waldmann
authors.forename: Letizia
authors.initials: L
authors.lastname: Boninsegna
authors.forename: Volker
authors.initials: V
authors.lastname: Fendrich
authors.forename: Peter K
authors.initials: PK
authors.lastname: Goretzki
authors.forename: Peter
authors.initials: P
authors.lastname: Langer
authors.forename: Peter H
authors.initials: PH
authors.lastname: Kann
authors.forename: Stefano
authors.initials: S
authors.lastname: Partelli
authors.forename: Detlef K
authors.initials: DK
authors.lastname: Bartsch
mesh_terms: D000293:Adolescent; D000328:Adult; D018572:Disease-Free Survival; D004379:Duodenal Neoplasms / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D005260:Female; D015408:Gastrinoma / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications; D016577:Pancreaticoduodenectomy; D055815:Young Adult