Pancreatic Neuroendocrine Tumors: Update on the New World Health Organization Classification

Gönter Klöppel, David S. Klimstra, Ralph H. Hruban, Volkan Adsay, Carlo Capella, Anne Couvelard, Paul Komminoth, Stefano La Rosa, Nobuyuki Ohike, Robert Y. Osamura, Aurel Perren, Jean Yves Scoazec, Guido Rindi

Research output: Contribution to journalReview articlepeer-review

30 Scopus citations

Abstract

The first classification of the pancreatic neuroendocrine neoplasms (PanNENs) that characterized the individual tumor by criteria with prognostic significance appeared in 1995. These criteria included a distinction by site and size, by degree of histological differentiation (well vs poor), by function (with and without hormonal syndrome), by angioinvasion, and by metastatic spread. The subsequently developedWorld Health Organization classifications in 2000, 2004, and 2010 largely followed this concept, but added proliferative activity as the best criterion reflecting tumor growth. In 2010, the classification combined histological differentiation with stratification into 3 tiers of proliferative activity, mainly using Ki67 as the most reliable measure of proliferation. The predictive value of this classification and grading system that was soon accompanied by an adequate TNM staging system has proved to be so excellent that all major treatment options of PanNENs, aswell as extrapancreatic NENs, have been currently based on this classification. The newWorld Health Organization 2017 classification is a refinement of the previous version. Its main change is the introduction of a "pancreatic neuroendocrine tumor grade 3" category to recognize grade-discordant pancreatic neuroendocrine tumors and distinguish them from pancreatic neuroendocrine carcinomas, which are defined by their poorly differentiated nature. There is increasing evidence that this phenotypical classification of PanNENs allows a targeted molecular analysis, which is going to broaden our understanding of the tumors' biology.

Original languageEnglish
Pages (from-to)233-239
Number of pages7
JournalAJSP: Reviews and Reports
Volume22
Issue number5
DOIs
StatePublished - 1 Sep 2017

Keywords

  • WHO 2017 classification
  • definition
  • grading
  • pancreatic neuroendocrine neoplasms

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