title: Metastasectomy of neuroendocrine tumors in patients with multiple endocrine neoplasia type 1.
abstract: BACKGROUND
  Neuroendocrine (NE) tumors commonly afflict patients with multiple endocrine neoplasia type 1 (MEN1). It is thought that patients with MEN1 have improved survival compared with individuals with analogous lesions. The role of metastasectomy of NE tumors in MEN1 patients is not clearly defined.
  
  
  METHODS
  A review of MEN1 patients undergoing surgery for NE tumors from 1994 to 2010 at a single tertiary care center was performed. Tumor function, the extent of metastasis, R0 resection, and survival were analyzed.
  
  
  RESULTS
  We identified 30 patients who underwent resection including synchronous and metachronous metastasectomy. Synchronous metastases were identified in 19 patients (63%), whereas 11 (37%) had metachronous disease. R0 resection was achieved in 93% of patients. Estimated 10-year survival is 86.4% (95% confidence interval, 60% to 100%) with no factors predictive of overall survival. The disease-free interval at 1, 5, and 10 years was 89%, 50%, and 19%, respectively, with recurrence occurring at a median of 5.4 years (95% confidence interval, 77.7% to 100%). Synchronous metastasis (P = .0072; hazard ratio [HR], 3.4) and nonfunctioning tumors (P = .014; HR, 3.3) were more likely to recur, whereas age (P = .09; HR, 1.5), gender (P = .49; HR, 1.3), and the site of metastasis (P = .81; HR, 1.1) did not influence recurrence.
  
  
  CONCLUSIONS
  Patients with MEN1 benefit from resection of metastatic NE disease. Despite a high recurrence rate, survival and disease-free interval is favorable vs patients without MEN1.
authors.affiliation: Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Benzon M
authors.initials: BM
authors.lastname: Dy
authors.affiliation: Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Florencia G
authors.initials: FG
authors.lastname: Que
authors.affiliation: Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Geoffrey B
authors.initials: GB
authors.lastname: Thompson
authors.affiliation: Department of Endocrinology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: William F
authors.initials: WF
authors.lastname: Young
authors.affiliation: Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Phillip
authors.initials: P
authors.lastname: Rowse
authors.affiliation: Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Veljko
authors.initials: V
authors.lastname: Strajina
authors.affiliation: Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address: richards.melanie@mayo.edu.
authors.forename: Melanie L
authors.initials: ML
authors.lastname: Richards
mesh_terms: D000328:Adult; D004379:Duodenal Neoplasms / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D059146:Metastasectomy; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000473:pathology* / Q000601:surgery*; D018358:Neuroendocrine Tumors / Q000473:pathology* / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000473:pathology* / Q000601:surgery*; D015996:Survival Rate; D016896:Treatment Outcome
keywords: MEN 1; Metastasectomy; Neuroendocrine tumor