Preprint Review Version 1 NOT YET PEER-REVIEWED

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

  1. Division of Cancer Genomics, National Cancer Center Research Institute, Tokyo 1040045, Japan
Version 1 : Received: 23 November 2016 / Approved: 24 November 2016 / Online: 24 November 2016 (10:59:33 CET)

How to cite: Ohmoto, A.; Rokutan, H.; Yachida, S. Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future. Preprints 2016, 2016110120 (doi: 10.20944/preprints201611.0120.v1). Ohmoto, A.; Rokutan, H.; Yachida, S. Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future. Preprints 2016, 2016110120 (doi: 10.20944/preprints201611.0120.v1).

Abstract

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 Ki-67 proliferation index and mitotic count according to the 2010 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 MEN1, DAXX and ATRX, whereas NECs have an inactivation of TP53 and RB1, 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 backround together with preclinical studies to develop new agents, including those already under investigation for SCLC, will be needed to improve the prognosis.

Subject Areas

pNENs; 2010 WHO classification; Ki-67 index; mitotic count; pNEC; tumor differentiation; whole-exome sequence data; everolimus; sunitinib; platinum regimen

Readers' Comments and Ratings (0)

Discuss and rate this article
Views 37
Downloads 39
Comments 0
Metrics 0
Discuss and rate this article

×
Alerts
Notify me about updates to this article or when a peer-reviewed version is published.