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BioMed Research International
Volume 2017 (2017), Article ID 9856140, 12 pages
https://doi.org/10.1155/2017/9856140
Review Article

Advances and Current Concepts in the Medical Management of Gastroenteropancreatic Neuroendocrine Neoplasms

1Clinic of Endocrine Oncology, Section of Endocrinology, Department of Pathophysiology, National and Kapodistrian University of Athens Medical School, Laiko University Hospital, Athens, Greece
2Department of Gastroenterology, Athens General Hospital “Georgios Gennimatas”, Athens, Greece

Correspondence should be addressed to Krystallenia I. Alexandraki; moc.liamg@kikardnaxela

Received 8 June 2017; Accepted 2 October 2017; Published 19 November 2017

Academic Editor: Peyman Björklund

Copyright © 2017 Krystallenia I. Alexandraki et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are rare and heterogeneous group of tumors presenting as localised or metastatic disease and in a subset with distinct clinical syndromes. Treatment is aimed at controlling the functional syndrome, eradicating the tumor, and/or preventing further tumor growth. Surgery is the treatment of choice in removing the primary tumor and/or reducing tumor burden but cannot be applied to all patients. Somatostatin analogs (SS-analogs) obtain control of functional syndromes in the majority of GEP-neuroendocrine tumors (NETs); phase III trials have shown that SS-analogs can be used as first-line antiproliferative treatment in patients with slow-growing GEP-NETs. The role of the recently approved serotonin inhibitor, telotristat ethyl, and gastrin receptor antagonist, netazepide, is evolving. Streptozotocin-based chemotherapy has been used for inoperable or progressing pancreatic NENs but the orally administered combination of capecitabine/temozolomide is becoming more popular due to its better tolerability and potential effect in other GEP-NENs. Phase III trials have shown efficacy of molecular targeted therapies in GEP-NETs and of radionuclide treatment in patients with midgut carcinoid tumors expressing somatostatin receptors. Most patients will develop disease progression necessitating further therapeutic options. A combination of currently available treatments along with the molecular signature of each tumor will guide future treatment.