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Review
. 2018 Jul 11:4:16.
doi: 10.1186/s40842-018-0066-3. eCollection 2018.

Current treatment strategies for patients with advanced gastroenteropancreatic neuroendocrine tumors (GEP-NETs)

Affiliations
Review

Current treatment strategies for patients with advanced gastroenteropancreatic neuroendocrine tumors (GEP-NETs)

Inbal Uri et al. Clin Diabetes Endocrinol. .

Abstract

Background: Neuroendocrine tumors (NETs) are rare neoplasms, with an estimated annual incidence of ~ 6.9/100,000. NETs arise throughout the body from cells of the diffuse endocrine system. More than half originate from endocrine cells of the gastrointestinal tract and the pancreas, thus being referred to as gastroenteropancreatic NETs (GEP NETs). The only treatment that offers a cure is surgery, however most patients are diagnosed with metastatic disease, and curative surgery is usually not an option.Since the majority of patients are not candidate for curative surgery, they can be offered long-term systemic treatment, for both symptomatic relief and tumor growth suppression. Evidence based treatment options include somatostatin analogues, everolimus (an mTOR inhibitor), sunitinib (a tyrosine kinase inhibitor), peptide receptor radionuclide therapy (PRRT), chemotherapy, etc., alone or combined with cytoreductive procedures (surgery or liver directed procedures). However, there is an increasing need for novel therapies. Other treatment options being investigated are immunotherapy and epigenetic assessment that may lead to more personalized interventions. Following first line therapy with somatostatin analogues, there is no clear information to date indicating a preferred treatment sequence, and therefore the treatment approach should be individualized based on each NET patient characteristics.

Conclusions: NET patients are increasingly diagnosed throughout the world, usually with metastatic disease and requiring systemic therapy. We believe that each patient should be therefore thoroughly evaluated and individually discussed by a multidisciplinary and dedicated NET-expert team, updated with all treatment options including ongoing clinical trials, and before selecting the proper treatment sequence.

Keywords: Neuroendocrine tumor; Treatment.

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Conflict of interest statement

Not applicable.Not applicable.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Ga68-DOTATATE-PET/CT images of a patient with metastatic G1 intestinal NET to the liver, bones and lymph nodes – showing high uptake be the tumor, in correlation with increased expression of somatostatin receptors, mainly SSTR2
Fig. 2
Fig. 2
Possible algorithm for treatment approach in patients with GEP-NETs

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