PMID- 29449689
OWN - NLM
STAT- MEDLINE
DCOM- 20190710
LR  - 20220129
IS  - 1759-5037 (Electronic)
IS  - 1759-5029 (Print)
IS  - 1759-5029 (Linking)
VI  - 14
IP  - 4
DP  - 2018 Apr
TI  - Current and emerging therapies for PNETs in patients with or without MEN1.
PG  - 216-227
LID - 10.1038/nrendo.2018.3 [doi]
AB  - Pancreatic neuroendocrine tumours (PNETs) might occur as a non-familial isolated 
      endocrinopathy or as part of a complex hereditary syndrome, such as multiple 
      endocrine neoplasia type 1 (MEN1). MEN1 is an autosomal dominant disorder 
      characterized by the combined occurrence of PNETs with tumours of the 
      parathyroids and anterior pituitary. Treatments for primary PNETs include 
      surgery. Treatments for non-resectable PNETs and metastases include biotherapy 
      (for example, somatostatin analogues, inhibitors of receptors and monoclonal 
      antibodies), chemotherapy and radiological therapy. All these treatments are 
      effective for PNETs in patients without MEN1; however, there is a scarcity of 
      clinical trials reporting the efficacy of the same treatments of PNETs in 
      patients with MEN1. Treatment of PNETs in patients with MEN1 is challenging owing 
      to the concomitant development of other tumours, which might have metastasized. 
      In recent years, preclinical studies have identified potential new therapeutic 
      targets for treating MEN1-associated neuroendocrine tumours (including PNETs), 
      and these include epigenetic modification, the beta-catenin-wingless (WNT) pathway, 
      Hedgehog signalling, somatostatin receptors and MEN1 gene replacement therapy. 
      This Review discusses these advances.
FAU - Frost, Morten
AU  - Frost M
AD  - Academic Endocrine Unit, Oxford Centre for Diabetes, Endocrinology and 
      Metabolism, Radcliffe Department of Medicine, University of Oxford, Churchill 
      Hospital, Oxford OX3 7LJ, UK.
AD  - Endocrine Research Unit, University of Southern Denmark, Sdr. Boulevard 29, 5000 
      Odense C, Denmark.
FAU - Lines, Kate E
AU  - Lines KE
AD  - Academic Endocrine Unit, Oxford Centre for Diabetes, Endocrinology and 
      Metabolism, Radcliffe Department of Medicine, University of Oxford, Churchill 
      Hospital, Oxford OX3 7LJ, UK.
FAU - Thakker, Rajesh V
AU  - Thakker RV
AD  - Academic Endocrine Unit, Oxford Centre for Diabetes, Endocrinology and 
      Metabolism, Radcliffe Department of Medicine, University of Oxford, Churchill 
      Hospital, Oxford OX3 7LJ, UK.
LA  - eng
GR  - G0601423/MRC_/Medical Research Council/United Kingdom
GR  - G1000467/MRC_/Medical Research Council/United Kingdom
GR  - 106995/WT_/Wellcome Trust/United Kingdom
GR  - G9825289/MRC_/Medical Research Council/United Kingdom
GR  - WT_/Wellcome Trust/United Kingdom
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20180216
PL  - England
TA  - Nat Rev Endocrinol
JT  - Nature reviews. Endocrinology
JID - 101500078
SB  - IM
MH  - Biological Therapy
MH  - Chemoprevention
MH  - Combined Modality Therapy
MH  - Epigenesis, Genetic
MH  - Genetic Therapy
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/genetics/*therapy
MH  - Neuroendocrine Tumors/*therapy
MH  - Pancreatic Neoplasms/*therapy
PMC - PMC6538535
MID - EMS83014
COIS- Disclosures: The authors declare that they have no competing interests as defined 
      by Springer Nature, or other interests that might be perceived to influence the 
      interpretation of the article.
EDAT- 2018/02/17 06:00
MHDA- 2019/07/11 06:00
PMCR- 2019/05/29
CRDT- 2018/02/17 06:00
PHST- 2018/02/17 06:00 [pubmed]
PHST- 2019/07/11 06:00 [medline]
PHST- 2018/02/17 06:00 [entrez]
PHST- 2019/05/29 00:00 [pmc-release]
AID - nrendo.2018.3 [pii]
AID - 10.1038/nrendo.2018.3 [doi]
PST - ppublish
SO  - Nat Rev Endocrinol. 2018 Apr;14(4):216-227. doi: 10.1038/nrendo.2018.3. Epub 2018 
      Feb 16.