Development and Validation of a Modified Eighth AJCC Staging System for Primary Pancreatic Neuroendocrine Tumors

Xu Feng Zhang, Feng Xue, Zheng Wu, Alexandra G. Lopez-Aguiar, George Poultsides, Eleftherios Makris, Flavio Rocha, Zaheer Kanji, Sharon Weber, Alexander Fisher, Ryan Fields, Bradley A. Krasnick, Kamran Idrees, Paula M. Smith, Cliff Cho, Megan Beems, Yi Lyu, Shishir K. Maithel, Timothy M. Pawlik

Research output: Contribution to journalArticlepeer-review

13 Scopus citations


Objective:To improve the prognostic accuracy of the eighth edition of AJCC staging system for pNETs with establishment and validation of a new staging system.Background:Validation of the updated eighth AJCC staging system for pNETs has been limited and controversial.Methods:Data from the SEER registry (1975-2016) (n = 3303) and a multi-institutional database (2000-2016) (n = 825) was used as development and validation cohorts, respectively. A mTNM was proposed by maintaining the eighth AJCC T and M definitions, and the recently proposed N status as N0 (no LNM), N1 (1-3 LNM), and N2 (≥4 LNM), but adopting a new stage classification.Results:The eighth TNM staging system failed to stratify patients with stage I versus IIA, stage IIB versus IIIA, and overall stage I versus II relative to long-Term OS in both database. There was a monotonic decrement in survival based on the proposed mTNM staging classification among patients derived from both the SEER (5-year OS, stage I 87.0% vs stage II 80.3% vs stage III 72.9% vs stage IV 57.2%, all P < 0.001), and multi-institutional (5-year OS, stage I 97.6% vs stage II 82.7% vs stage III 78.4% vs stage IV 50.0%, all P < 0.05) datasets. On multivariable analysis, mTNM staging remained strongly associated with prognosis, as the hazard of death incrementally increased with each stage among patients in the 2 cohorts.Conclusion:A mTNM pNETs clinical staging system using N0, N1, N2 nodal categories was better at stratifying patients relative to long-Term OS than the eighth AJCC staging.

Original languageEnglish
Pages (from-to)E773-E780
JournalAnnals of surgery
Issue number6
StatePublished - Jun 1 2022


  • AJCC
  • Neuroendocrine tumor
  • Pancreas
  • Prognosis
  • Staging manual


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