title: Enucleation and limited pancreatic resection provide long-term cure for insulinoma in multiple endocrine neoplasia type 1.
abstract: OBJECTIVE
  To assess the characteristics and long-term outcome after surgery in patients with multiple endocrine neoplasia type 1 (MEN1)-associated insulinoma.
  
  
  METHODS
  Retrospective analysis of prospectively collected data of MEN1 patients with organic hyperinsulinism at a tertiary referral center.
  
  
  RESULTS
  Thirteen (17%) of 74 patients with MEN1 had organic hyperinsulinism. The median age at diagnosis was 27 (range 9-48) years. In 7 patients insulinoma was the first manifestation of the syndrome. All patients had at least one pancreatic neuroendocrine neoplasm (pNEN) upon imaging, including CT, MRI or endoscopic ultrasonography. Seven patients had solitary lesions upon imaging, 4 patients had one dominant tumor with coexisting multiple small pNENs, and 2 patients had multiple lesions without dominance. Eight patients had limited resections (1 segmental resection, 7 enucleations), 4 subtotal distal pancreatectomies, and 1 patient a partial duodenopancreatectomy. There was no postoperative mortality. Six patients experienced complications, including pancreatic fistula in 5 patients. Pathological examination revealed median three (range 1-14) macro-pNENs sized between 6 and 40 mm, and a total of 14 potentially benign insulinomas were detected in the 13 patients. After median follow-up of 156 months, only 1 patient developed recurrent hyperinsulinism after initial enucleation. Twelve patients developed new pNENs in the pancreatic remnant and 4 patients underwent reoperations (3 for metastatic ZES, 1 for recurrent hyperinsulinism). One of 5 patients with an initial extended pancreatic resection developed insulin-dependent diabetes mellitus.
  
  
  CONCLUSIONS
  Enucleation and limited resection provide long-term cure for MEN1 insulinoma in patients with solitary or dominant tumors. Subtotal distal pancreatectomy should thus be preserved for patients with multiple pNENs without dominance given the risk of exocrine and endocrine pancreas insufficiency in the mostly young patients.
authors.affiliation: Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Marburg, Germany.
authors.forename: Detlef K
authors.initials: DK
authors.lastname: Bartsch
authors.forename: Max
authors.initials: M
authors.lastname: Albers
authors.forename: Richard
authors.initials: R
authors.lastname: Knoop
authors.forename: Peter H
authors.initials: PH
authors.lastname: Kann
authors.forename: Volker
authors.initials: V
authors.lastname: Fendrich
authors.forename: Jens
authors.initials: J
authors.lastname: Waldmann
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007340:Insulinoma / Q000473:pathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000473:pathology / Q000601:surgery*; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D010180:Pancreatectomy / Q000009:adverse effects / Q000379:methods; D010185:Pancreatic Fistula / Q000209:etiology; D010190:Pancreatic Neoplasms / Q000473:pathology / Q000601:surgery*; D011446:Prospective Studies; D012086:Reoperation; D012189:Retrospective Studies; D062606:Tertiary Care Centers; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult