Open AccessModels of Gastroenteropancreatic Neuroendocrine Neoplasms: Current Status and Future Directions
Author(s) -
Katharina Detjen,
Linda Hammerich,
Burcin Özdirik,
Münevver Demir,
Bertram Wiedenmann,
Frank Tacke,
Henning Jann,
Christoph Roderburg
Publication year - 2020
Publication title -
neuroendocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.493
H-Index - 101
eISSN - 1423-0194
pISSN - 0028-3835
DOI - 10.1159/000509864
Subject(s) - neuroendocrine tumors , context (archaeology) , disease , clinical trial , pancreas , bioinformatics , gastrointestinal tract , endocrine system , biology , medicine , pathology , oncology , hormone , paleontology
Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are a rare, heterogeneous group of tumors that originate from the endocrine system of the gastrointestinal tract and pancreas. GEP-NENs are subdivided according to their differentiation into well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). Since GEP-NENs represent rare diseases, only limited data from large prospective, randomized clinical trials are available, and recommendations for treatment of GEP-NEN are in part based on data from retrospective analyses or case series. In this context, tractable disease models that reflect the situation in humans and that allow to recapitulate the different clinical aspects and disease stages of GEP-NET or GEP-NEC are urgently needed. In this review, we highlight available data on mouse models for GEP-NEN. We discuss how these models reflect tumor biology of human disease and whether these models could serve as a tool for understanding the pathogenesis of GEP-NEN and for disease modeling and pharmacosensitivity assays, facilitating prediction of treatment response in patients. In addition, open issues applicable for future developments will be discussed.