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A Single Institution's 26-Year Experience With Nonfunctional Pancreatic Neuroendocrine Tumors: A Validation of Current Staging Systems and a New Prognostic Nomogram

Ellison, Trevor A. MD*; Wolfgang, Christopher L. MD, PhD*; Shi, Chanjuan MD, PhD§; Cameron, John L. MD*; Murakami, Peter ScM; Mun, Liew Jun MBBS; Singhi, Aatur D. MD, PhD; Cornish, Toby C. MD, PhD; Olino, Kelly MD*; Meriden, Zina MD; Choti, Michael MD, MBA*,‡; Diaz, Luis A. MD; Pawlik, Timothy M. MD, MPH*; Schulick, Richard D. MD; Hruban, Ralph H. MD†,‡; Edil, Barish H. MD

Annals of Surgery:
doi: 10.1097/SLA.0b013e31828f3174
Features
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.

In Brief

This article utilizes a single institution's 26-year experience with nonsyndromic, nonfunctional pancreatic neuroendocrine tumors (PanNETs) to validate the 2010 AJCC (American Joint Committee on Cancer) and 2006 ENETS (European Neuroendocrine Tumor Society) tumor staging systems for PanNETs. Furthermore, the article develops a new, simple, and more accurate prognostic tool for nonsyndromic, nonfunctional PanNETs.

Author Information

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.

Reprints: Barish H. Edil, MD, Department of Surgery, University of Colorado, 1665 Aurora Court, Room 3337, Aurora, CO 80045. E-mail: barish.edil@ucdenver.edu.

No support has been taken from any source.

Disclosure: The authors declare no conflicts of interest.

© 2014 by Lippincott Williams & Wilkins.