title: Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours.
abstract: BACKGROUND
  In patients with multiple endocrine neoplasia type 1 (MEN-1), pancreaticoduodenal (PD) neuroendocrine tumours (NETs) are associated with early mortality, yet the best treatment strategy remains uncertain.
  
  
  OBJECTIVE
  To assess patient important outcomes (mortality and metastasis) of PD-NETs and predictors of outcomes in patients with MEN-1.
  
  
  METHODS
  Retrospective cohort of patients with MEN-1 who attended the Mayo Clinic, Rochester, MN from 1997 to 2014.
  
  
  RESULTS
  We identified 287 patients with MEN-1; 199 (69%) patients had 217 PD-NETs. Among those with a PD-NETs, 129 (65%) had surgery of which 90 (70%) had their primary surgery performed at Mayo Clinic. The median postoperative follow-up was 8 years during which 13 (14%) patients died. The mean (±standard deviation) age of death was 51 (±9) years. Tumour size, metastasis at surgery or tumour type were not predictive of mortality, but for every year older at surgery, the odds of metastasis increased by 6%. Surgery was not performed in 70 (35%) patients. Among those who were observed/medically managed without known metastatic disease, mean tumour growth was 0·02 cm/year (range, -0·13-0·4 cm/year). Four patients (7%) died at a median age of 77 (range, 51-89) years.
  
  
  CONCLUSIONS
  PD-NETs are common in patients with MEN-1 and are associated with early mortality even after surgical intervention. Active surveillance is a viable option in nonaggressive PD-NETs, although definitive factors identifying such patients are lacking. Therefore, counselling regarding risks and benefits of current treatment options remains integral to the care of patients with MEN-1.
authors: Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA.
authors: D
authors: D
authors: Donegan
authors: Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA.|Knowledge and Evaluation Research Unit in Endocrinology (KER-Endo), Division of Endocrinology, Diabetes, Metabolism and Nutrition, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
authors: N
authors: N
authors: Singh Ospina
authors: Knowledge and Evaluation Research Unit in Endocrinology (KER-Endo), Division of Endocrinology, Diabetes, Metabolism and Nutrition, Department of Medicine, Mayo Clinic, Rochester, MN, USA.|Division of Endocrinology, Department of Internal Medicine, University Hospital "Dr. Jose E. Gonzalez", Autonomous University of Nuevo Leon, Monterrey, Mexico.
authors: R
authors: R
authors: Rodriguez-Gutierrez
authors: Division of Surgery, Mayo Clinic, Rochester, MN, USA.
authors: Z
authors: Z
authors: Al-Hilli
authors: Division of Surgery, Mayo Clinic, Rochester, MN, USA.
authors: G B
authors: GB
authors: Thompson
authors: Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA.
authors: B L
authors: BL
authors: Clarke
authors: Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA.
authors: W F
authors: WF
authors: Young
mesh_terms: D000328:Adult; D015331:Cohort Studies; D005500:Follow-Up Studies; D006801:Humans; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications* / Q000401:mortality / Q000473:pathology; D009362:Neoplasm Metastasis; D018358:Neuroendocrine Tumors / Q000150:complications* / Q000401:mortality / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000737:chemistry* / Q000401:mortality / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult