Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=29590641
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Gastroenteropancreatic neuroendocrine neoplasms: genes, therapies and models
#MMPMID29590641
Kawasaki K
; Fujii M
; Sato T
Dis Model Mech
2018[Feb]; 11
(2
): ? PMID29590641
show ga
Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) refer to a group of
heterogeneous cancers of neuroendocrine cell phenotype that mainly fall into one
of two subtypes: gastroenteropancreatic neuroendocrine tumors (GEP-NETs; well
differentiated) or gastroenteropancreatic neuroendocrine carcinomas (GEP-NECs;
poorly differentiated). Although originally defined as orphan cancers, their
steadily increasing incidence highlights the need to better understand their
etiology. Accumulating epidemiological and clinical data have shed light on the
pathological characteristics of these diseases. However, the relatively low
number of patients has hampered conducting large-scale clinical trials and hence
the development of novel treatment strategies. To overcome this limitation,
tractable disease models that faithfully reflect clinical features of these
diseases are needed. In this Review, we summarize the current understanding of
the genetics and biology of these diseases based on conventional disease models,
such as genetically engineered mouse models (GEMMs) and cell lines, and discuss
the phenotypic differences between the models and affected humans. We also
highlight the emerging disease models derived from human clinical samples,
including patient-derived xenograft models and organoids, which may provide
biological and therapeutic insights into GEP-NENs.
free
free
free
