title: Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1.
abstract: OBJECTIVE
  To evaluate the outcome of an aggressive surgical approach for duodenopancreatic neuroendocrine tumors (PETs) associated with multiple endocrine neoplasia type 1 (MEN1).
  
  
  BACKGROUND
  The management of PETs is still controversial in the setting of the autosomal dominant inherited MEN1 syndrome.
  
  
  METHODS
  MEN1 patients that had either biochemical evidence of functioning PETs or visualized nonfunctioning PETs larger than 1 cm in size on imaging were operated. Since 1997, patients were followed annually by biochemical testing and imaging studies.
  
  
  RESULTS
  Twenty-six genetically confirmed MEN1 patients underwent duodenopancreatic resection for functioning (n = 17) or nonfunctioning (n = 9) PETs. Ten (38%) patients had malignant PETs as characterized by the presence of lymph node (10 patients) and/or distant metastases (2 patients). The surgical approach was selected based on the type, location, and size of PETs. Four Zollinger-Ellison syndrome (ZES) patients required pylorus preserving pancreaticoduodenectomy (PPPD) as initial or redo procedure, 20 patients underwent other duodenopancreatic resections, and 2 patients had simple enucleations of PETs. After median 83 months (range, 5-241 months), 24 patients were alive and 2 patients died of an unrelated cause. All patients with insulinoma or vipoma and 7 of 11 patients with ZES were biochemically cured, including the ZES patients who underwent PPPD. However, 19 of 26 (73%) patients developed new small PETs (<1 cm) in the pancreatic remnant, but no patient had yet detectable metastases on imaging.
  
  
  CONCLUSIONS
  Early and aggressive surgery of PETs in MEN1 patients prevents the development of liver metastases, which are the most life-threatening determinant. PPPD might be the procedure of choice for MEN1-ZES, which has to be proven in large scale studies.
authors.affiliation: Department of Surgery, Division of Endocrinology, Philipps-University Marburg, Baldingerstrasse, D-35043 Marburg, Germany. bartsch@mailer.uni-marburg.de
authors.forename: Detlef K
authors.initials: DK
authors.lastname: Bartsch
authors.forename: Volker
authors.initials: V
authors.lastname: Fendrich
authors.forename: Peter
authors.initials: P
authors.lastname: Langer
authors.forename: Ilhan
authors.initials: I
authors.lastname: Celik
authors.forename: Peter H
authors.initials: PH
authors.lastname: Kann
authors.forename: Matthias
authors.initials: M
authors.lastname: Rothmund
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016009:Chi-Square Distribution; D004252:DNA Mutational Analysis; D005260:Female; D015408:Gastrinoma / Q000473:pathology / Q000601:surgery; D006801:Humans; D007340:Insulinoma / Q000473:pathology / Q000601:surgery; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D009364:Neoplasm Recurrence, Local; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D016577:Pancreaticoduodenectomy / Q000379:methods*; D011183:Postoperative Complications; D011446:Prospective Studies; D018709:Statistics, Nonparametric; D016896:Treatment Outcome; D003969:Vipoma / Q000473:pathology / Q000601:surgery