title: A single institution's 26-year experience with nonfunctional pancreatic neuroendocrine tumors: a validation of current staging systems and a new prognostic nomogram.
abstract: OBJECTIVE
  To validate the 2010 American Joint Committee on Cancer (AJCC) and 2006 European Neuroendocrine Tumor Society (ENETS) tumor staging systems for pancreatic neuroendocrine tumors (PanNETs) using the largest, single-institution series of surgically resected patients in the literature.
  
  
  BACKGROUND
  The natural history and prognosis of PanNETs have been poorly defined because of the rarity and heterogeneity of these neoplasms. Currently, there are 2 main staging systems for PanNETs, which can complicate comparisons of reports in the literature and thereby hinder progress against this disease.
  
  
  METHODS
  Univariate and multivariate analyses were conducted on the prognostic factors of survival using 326 sporadic, nonfunctional, surgically resected PanNET patients who were cared for at our institution between 1984 and 2011. Current and proposed models were tested for survival prognostication validity as measured by discrimination (Harrel's c-index, HCI) and calibration.
  
  
  RESULTS
  Five-year overall-survival rates for AJCC stages I, II, and IV are 93% (88%-99%), 74% (65%-83%), and 56% (42%-73%), respectively, whereas ENETS stages I, II, III, and IV are 97% (92%-100%), 87% (80%-95%), 73% (63%-84%), and 56% (42%-73%), respectively. Each model has an HCI of 0.68, and they are no different in their ability to predict survival. We developed a simple prognostic tool just using grade, as measured by continuous Ki-67 labeling, sex, and binary age that has an HCI of 0.74.
  
  
  CONCLUSIONS
  Both the AJCC and ENETS staging systems are valid and indistinguishable in their survival prognostication. A new, simpler prognostic tool can be used to predict survival and decrease interinstitutional mistakes and uncertainties regarding these neoplasms.
authors.affiliation: Departments of *Surgery †Pathology ‡Oncology, The Sol Goldman Pancreatic Cancer Research Center, the Johns Hopkins University School of Medicine, Sidney Kimmel Comprehensive Cancer Center, Baltimore, MD §Vanderbilt University Medical Center, Department of Pathology, Microbiology and Immunology, Nashville, TN ¶The Johns Hopkins Bloomberg School of Public Health, Department of Biostatistics, Baltimore, MD; and ∥The University of Colorado, Department of Surgery, Aurora, CO.
authors.forename: Trevor A
authors.initials: TA
authors.lastname: Ellison
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Wolfgang
authors.forename: Chanjuan
authors.initials: C
authors.lastname: Shi
authors.forename: John L
authors.initials: JL
authors.lastname: Cameron
authors.forename: Peter
authors.initials: P
authors.lastname: Murakami
authors.forename: Liew Jun
authors.initials: LJ
authors.lastname: Mun
authors.forename: Aatur D
authors.initials: AD
authors.lastname: Singhi
authors.forename: Toby C
authors.initials: TC
authors.lastname: Cornish
authors.forename: Kelly
authors.initials: K
authors.lastname: Olino
authors.forename: Zina
authors.initials: Z
authors.lastname: Meriden
authors.forename: Michael
authors.initials: M
authors.lastname: Choti
authors.forename: Luis A
authors.initials: LA
authors.lastname: Diaz
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Pawlik
authors.forename: Richard D
authors.initials: RD
authors.lastname: Schulick
authors.forename: Ralph H
authors.initials: RH
authors.lastname: Hruban
authors.forename: Barish H
authors.initials: BH
authors.lastname: Edil
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003661:Decision Support Techniques; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D060787:Neoplasm Grading; D009367:Neoplasm Staging; D018358:Neuroendocrine Tumors / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D049451:Nomograms; D010190:Pancreatic Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D015203:Reproducibility of Results; D016019:Survival Analysis; D047368:Tumor Burden