Why did they change that? Practical implications of the evolving classification of neuroendocrine tumours of the gastrointestinal tract

Abstract

Neuroendocrine neoplasms (NENs) of the gastrointestinal tract (GIT) comprise neuroendocrine tumours (NETs) and neuroendocrine carcinomas (NECs). During the last decade the classification and grading of GIT NENs has undergone significant changes, culminating in the World Health Organisation (WHO) 2019 classification. These changes, some of which are attributable to an only partially successful attempt to achieve uniform nomenclature among different organs, include: slight changes to the cut-off used for the Ki-67 proliferative index to distinguish grade 1 from grade 2 NETs; an emphasis on the distinction between grade 3 NETs (low-grade NETs with a high proliferative rate) and NECs which, by definition, are all high grade; classification of tumours with mixed non-neuroendocrine and neuroendocrine differentiation as MiNENs; and replacement of the term 'goblet cell carcinoid' with 'goblet cell adenocarcinoma'. While some of these changes seem minor, even semantic, each was made for very specific reasons which reflect an improved understanding of neuroendocrine neoplasia. The changes have definite implications for pathologists in clinical practice, not all of which may be readily apparent. This review is an attempt to explain the background behind each of the recent changes to the classification of neuroendocrine neoplasms of the gastrointestinal tract and summarise their impact on surgical pathologists - including a guide on how to approach certain recurrent difficulties encountered with the WHO 2019 system in routine clinical practice.

Keywords: Ki-67 proliferative index; NEC; neuroendocrine tumour.