Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future

Pancreatic neuroendocrine neoplasms (pNENs) are rare tumors accounting for only 1%-2% of all pancreatic tumors. pNENs are pathologically heterogeneous and are categorized into three groups (neuroendocrine tumor: NET G1, NET G2; and neuroendocrine carcinoma: NEC) on the basis of the Ki-67 proliferati...

Full description

Saved in:
Bibliographic Details
Published in:International journal of molecular sciences 2017-01, Vol.18 (1), p.143-143
Main Authors: Ohmoto, Akihiro, Rokutan, Hirofumi, Yachida, Shinichi
Format: Article
Language:English
Subjects:
2010 WHO classification
Carcinoma, Neuroendocrine - pathology
Carcinoma, Neuroendocrine - therapy
Cell Differentiation
Classification
everolimus
Humans
Ki-67 index
mitotic count
Models, Biological
Pancreatic cancer
Pancreatic Neoplasms - genetics
Pancreatic Neoplasms - pathology
Pancreatic Neoplasms - therapy
platinum regimen
pNEC
pNENs
Review
Signal Transduction
sunitinib
tumor differentiation
Tumors
whole-exome sequence data
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Pancreatic neuroendocrine neoplasms (pNENs) are rare tumors accounting for only 1%-2% of all pancreatic tumors. pNENs are pathologically heterogeneous and are categorized into three groups (neuroendocrine tumor: NET G1, NET G2; and neuroendocrine carcinoma: NEC) on the basis of the Ki-67 proliferation index and the mitotic count according to the 2010 World Health Organization (WHO) classification of gastroenteropancreatic NENs. NEC in this classification includes both histologically well-differentiated and poorly differentiated subtypes, and modification of the WHO 2010 classification is under discussion based on genetic and clinical data. Genomic analysis has revealed NETs G1/G2 have genetic alterations in chromatin remodeling genes such as , and , whereas NECs have an inactivation of and , and these data suggest that different treatment approaches would be required for NET G1/G2 and NEC. While there are promising molecular targeted drugs, such as everolimus or sunitinib, for advanced NET G1/G2, treatment stratification based on appropriate predictive and prognostic biomarkers is becoming an important issue. The clinical outcome of NEC is still dismal, and a more detailed understanding of the genetic background together with preclinical studies to develop new agents, including those already under investigation for small cell lung cancer (SCLC), will be needed to improve the prognosis.
ISSN:1422-0067
1661-6596
1422-0067
DOI:10.3390/ijms18010143