Gastro-entero-pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: a therapy update

Gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) are the second most common tumors in multiple endocrine neoplasia type 1 (MEN1), mainly occurring in pancreatic islets and duodenum, usually as multiple tumors. They can manifest as both nonfunctioning and functioning tumors. Currently, surgi...

Full description

Saved in:
Bibliographic Details
Published inInternational journal of endocrine oncology Vol. 4; no. 1; pp. 43 - 58
Main Authors Tonelli, Francesco, Marini, Francesca, Giusti, Francesca, Brandi, Maria Luisa
Format Journal Article
LanguageEnglish
Published London Future Medicine Ltd 01.03.2017
Subjects
Clinical trials
genetic test
GEP-NETs
Localization
Medical imaging
medical therapy
MEN1
Metastasis
Mutation
Neuroendocrine tumors
NMR
Nuclear magnetic resonance
Pancreatic cancer
Patients
Polypeptides
Proteins
surgical therapy
Tumorigenesis
Online AccessGet full text

Cover

More Information
Summary:Gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) are the second most common tumors in multiple endocrine neoplasia type 1 (MEN1), mainly occurring in pancreatic islets and duodenum, usually as multiple tumors. They can manifest as both nonfunctioning and functioning tumors. Currently, surgical removal of GEP-NETs in MEN1 represents the gold standard curative approach. Conventional medical therapies for sporadic GEP-NETs showed to be effective also in a percentage of MEN1 patients. Innovative medical therapies, that have demonstrated to be effective on sporadic GEP-NETs, still need to be evaluated on MEN1 patients in prospective clinical trials and long-term follow-up. This review resumes current knowledge of MEN1 GEP-NETs, discussing surgical and medical approaches, genetic and molecular bases of tumorigenesis, and presenting novel possible drug therapies.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 14
ISSN:2045-0869
2045-0877
DOI:10.2217/ije-2016-0017