title: Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1: surgery or surveillance?
abstract: BACKGROUND
  The management of pancreaticoduodenal endocrine tumors (PETs) remains controversial in multiple endocrine neoplasia type 1 (MEN 1).
  
  
  METHODS
  Twenty-one patients with MEN 1 and PETs were analyzed for outcome of surgery and surveillance with special regard to the genotype based on MEN1 gene mutation analysis.
  
  
  RESULTS
  Nine patients had gastrinomas, 5 had nonfunctioning tumors, 4 had insulinomas, 2 had insulinomas and gastrinomas, and 1 had a VIPoma. Seven patients (33%) had malignant tumors. Sixteen patients (76%) were initially treated by pancreatic resections or tumor enucleations or both. Six patients underwent reoperations for recurrences or lymph node metastases or both. Fifteen of the 16 operated patients are alive, and 12 have no evidence of disease after a median follow-up of 78 months (range, 1-198 months). Five patients with gastrinomas or nonfunctioning tumors, but no symptoms, underwent surveillance; 1 of them developed lymph node metastases. Patients with truncating mutations in the N- or C-terminal region (exons 2, 9, or 10) of the MEN1 gene had a significantly higher rate of malignant tumors (55% vs 10%; P <.05) than patients with other mutations.
  
  
  CONCLUSIONS
  An aggressive surgical approach is justified for PETs in patients with MEN 1. However, MEN1 gene mutations in exons 3 to 8 seem to be associated with mild behavior of PETs, possibly allowing surveillance in asymptomatic patients.
authors: Department of Surgery, Philipps-University Marburg, Germany.
authors: D K
authors: DK
authors: Bartsch
authors: P
authors: P
authors: Langer
authors: A
authors: A
authors: Wild
authors: T
authors: T
authors: Schilling
authors: I
authors: I
authors: Celik
authors: M
authors: M
authors: Rothmund
authors: C
authors: C
authors: Nies
mesh_terms: D000328:Adult; D004379:Duodenal Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D005260:Female; D005838:Genotype; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D009154:Mutation; D009363:Neoplasm Proteins / Q000235:genetics; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D011518:Proto-Oncogene Proteins
chemical_list: C105373:MEN1 protein, human; D009363:Neoplasm Proteins; D011518:Proto-Oncogene Proteins