Publication: Pancreatic neuroendocrine neoplasms:
Clinicopathological features and pathological staging
Authors
Lam, Alfred King-yin ; Ishida, Hirotaka
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Publisher
Universidad de Murcia, Departamento de Biologia Celular e Histiologia
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DOI
https://doi.org/10.14670/HH-18-288
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info:eu-repo/semantics/article
Description
Abstract
. The nomenclature and classification of
pancreatic neuroendocrine neoplasms has evolved in the
last 15 years based on the advances in knowledge of the
genomics, clinical behaviour and response to therapies.
The current 2019 World Health Organization
classification of pancreatic neuroendocrine neoplasms
categorises them into three groups; pancreatic
neuroendocrine tumours (PanNETs) (grade 1 grade 2,
grade 3), pancreatic neuroendocrine carcinomas and
mixed neuroendocrine-non-neuroendocrine neoplasms
(MiNENs) based on the mitotic rate, Ki-67 index,
morphological differentiation and/or co-existing tissue
subtype. PanNETs are also classified into non-functional
NET, insulinoma, gastrinoma, VIPoma, glucagonoma,
somatostatinoma, ACTH-producing NET and serotonin
producing NET based on hormone production and
clinical manifestations. A portion of the cases were
associated with genetic syndromes such as multiple
neuroendocrine neoplasia 1 (MEN 1), neurofibromatosis
and Von Hippel-Lindau syndrome. In view of the
distinctive pathology and clinical behaviour of
PanNENs, the current 8th AJCC/UICC staging system
has separated prognostic staging grouping for PanNETs
from the pancreatic neuroendocrine carcinomas or
MiNENs. Pancreatic neuroendocrine carcinomas and
MiNENs are staged according to the prognostic stage
grouping for exocrine pancreatic carcinoma. The new
stage grouping of PanNETs was validated to have
survival curves separated between different prognostic
groups. This refined histological and staging would lead
to appropriate selections of treatment strategies for the
patients with pancreatic neuroendocrine neoplasms
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Citation
Histology and Histopathology Vol. 36, nĀŗ4 (2021)
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