PMID- 31443481
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200929
IS  - 2077-0383 (Print)
IS  - 2077-0383 (Electronic)
IS  - 2077-0383 (Linking)
VI  - 8
IP  - 9
DP  - 2019 Aug 22
TI  - Updates on the Role of Molecular Alterations and NOTCH Signalling in the 
      Development of Neuroendocrine Neoplasms.
LID - 10.3390/jcm8091277 [doi]
LID - 1277
AB  - Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of rare 
      malignancies, mainly originating from hormone-secreting cells, which are 
      widespread in human tissues. The identification of mutations in ATRX/DAXX genes 
      in sporadic NENs, as well as the high burden of mutations scattered throughout 
      the multiple endocrine neoplasia type 1 (MEN-1) gene in both sporadic and 
      inherited syndromes, provided new insights into the molecular biology of tumour 
      development. Other molecular mechanisms, such as the NOTCH signalling pathway, 
      have shown to play an important role in the pathogenesis of NENs. NOTCH receptors 
      are expressed on neuroendocrine cells and generally act as tumour suppressor 
      proteins, but in some contexts can function as oncogenes. The biological 
      heterogeneity of NENs suggests that to fully understand the role and the 
      potential therapeutic implications of gene mutations and NOTCH signalling in 
      NENs, a comprehensive analysis of genetic alterations, NOTCH expression patterns 
      and their potential role across all NEN subtypes is required.
FAU - von Arx, Claudia
AU  - von Arx C
AUID- ORCID: 0000-0001-6078-4271
AD  - Department of Abdominal Oncology, Istituto Nazionale Tumori, IRCCS Fondazione "G. 
      Pascale", 80131 Naples, Italy.
AD  - Department of Surgery and Cancer, Imperial College London, London W12 0HS, UK.
FAU - Capozzi, Monica
AU  - Capozzi M
AD  - Department of Abdominal Oncology, Istituto Nazionale Tumori, IRCCS Fondazione "G. 
      Pascale", 80131 Naples, Italy.
FAU - Lopez-Jimenez, Elena
AU  - Lopez-Jimenez E
AD  - Cancer Cell Metabolism Group. Centre for Haematology, Immunology and Inflammation 
      Department, Imperial College London, London W12 0HS, UK.
FAU - Ottaiano, Alessandro
AU  - Ottaiano A
AD  - SSD Innovative Therapies for Abdominal Metastases-Department of Abdominal 
      Oncology, Istituto Nazionale Tumori, IRCCS-Fondazione "G. Pascale", 80131 Naples, 
      Italy.
FAU - Tatangelo, Fabiana
AU  - Tatangelo F
AUID- ORCID: 0000-0002-9943-6079
AD  - Department of Pathology, Istituto Nazionale Tumori, IRCCS-Fondazione "G. 
      Pascale", 80131 Naples, Italy.
FAU - Di Mauro, Annabella
AU  - Di Mauro A
AD  - Department of Pathology, Istituto Nazionale Tumori, IRCCS-Fondazione "G. 
      Pascale", 80131 Naples, Italy.
FAU - Nasti, Guglielmo
AU  - Nasti G
AD  - SSD Innovative Therapies for Abdominal Metastases-Department of Abdominal 
      Oncology, Istituto Nazionale Tumori, IRCCS-Fondazione "G. Pascale", 80131 Naples, 
      Italy.
FAU - Tornesello, Maria Lina
AU  - Tornesello ML
AUID- ORCID: 0000-0002-3523-3264
AD  - Unit of Molecular Biology and Viral Oncology, Department of Research, Istituto 
      Nazionale Tumori IRCCS Fondazione Pascale, 80131 Naples, Italy. 
      m.tornesello@istitutotumori.na.it.
FAU - Tafuto, Salvatore
AU  - Tafuto S
AD  - Department of Abdominal Oncology, Istituto Nazionale Tumori, IRCCS Fondazione "G. 
      Pascale", 80131 Naples, Italy. s.tafuto@istitutotumori.na.it.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20190822
PL  - Switzerland
TA  - J Clin Med
JT  - Journal of clinical medicine
JID - 101606588
PMC - PMC6780206
OTO - NOTNLM
OT  - NOTCH
OT  - cancer-driven genes
OT  - germline mutations
OT  - malignant castration-resistant prostatic cells
OT  - medullary thyroid carcinoma
OT  - mutational mechanism
OT  - neuroendocrine neoplasms
OT  - pancreatic NET
OT  - small bowel NET
OT  - small cell lung carcinoma
COIS- The authors declare no conflict of interest.
EDAT- 2019/08/25 06:00
MHDA- 2019/08/25 06:01
PMCR- 2019/08/22
CRDT- 2019/08/25 06:00
PHST- 2019/07/10 00:00 [received]
PHST- 2019/08/13 00:00 [revised]
PHST- 2019/08/20 00:00 [accepted]
PHST- 2019/08/25 06:00 [entrez]
PHST- 2019/08/25 06:00 [pubmed]
PHST- 2019/08/25 06:01 [medline]
PHST- 2019/08/22 00:00 [pmc-release]
AID - jcm8091277 [pii]
AID - jcm-08-01277 [pii]
AID - 10.3390/jcm8091277 [doi]
PST - epublish
SO  - J Clin Med. 2019 Aug 22;8(9):1277. doi: 10.3390/jcm8091277.