title: Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate.
abstract: Distal pancreatectomy is the most frequent operation for insulinomas complicating multiple endocrine neoplasia type 1 insulinoma, although there are conditions for which a different operative approach might be preferable. In this article, we report the operative experience of a referral center for multiple endocrine neoplasia type 1 insulinoma.
  
  
  
  Twelve patients underwent operations between 1992 and 2015: 8 underwent a distal pancreatic resection, and 4 underwent a pancreatoduodenectomy. Enucleation of other macroadenomas present in the remnant pancreas was performed in 9 out of these 12 patients.
  
  
  
  Operative complications (2 pancreatic fistulas and 2 cases of pancreatitis) occurred in 4 of the 8 distal pancreatic resections. In 1 patient, reoperation was required to resolve the complications of the first operation. At pathologic analysis, multiple insulinomas were found in 5 patients, lymph-nodal metastasis positive for insulin in 2 patients, multiple nonfunctioning pancreatic tumors in all patients, glucagonoma in 4 patients, and gastrinoma in the duodenum or lymph nodes in 4 patients. All the patients were treated successfully for the hypoglycemic/hyperinsulinemic syndrome with no clinical recurrence at a mean follow-up of 85 months (range 4-242 months). Recurrent nonfunctioning pancreatic tumor macroadenomas in the remnant pancreas occurred in only 1 of the 12 patients, and no progression of the gastrinomas was observed. None of the patients developed diabetes mellitus.
  
  
  
  Resection of the most severely affected part of the pancreas, whether left or right, associated with enucleation of concomitant macroadenomas in the preserved pancreas is recommended for the treatment of hypoglycemic/hyperinsulinemic syndrome and to prevent malignant progression of nonfunctioning pancreatic tumors in patients with multiple endocrine neoplasia type 1. If the head of the pancreas is the most affected site and the Zollinger-Ellison syndrome is concomitant, then pancreatoduodenectomy should be preferred over distal pancreatectomy.
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. Electronic address: francesco.tonelli@unifi.it.
authors.forename: Francesco
authors.initials: F
authors.lastname: Tonelli
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Giudici
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Nesi
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Batignani
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Brandi
mesh_terms: D000328:Adult; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007340:Insulinoma / Q000473:pathology / Q000601:surgery*; D008297:Male; D018761:Multiple Endocrine Neoplasia Type 1 / Q000473:pathology / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000473:pathology / Q000601:surgery*; D016577:Pancreaticoduodenectomy; D018579:Patient Selection; D016896:Treatment Outcome