Update on Management of Midgut Neuroendocrine Tumors

Abstract

Midgut neuroendocrine tumors are typically indolent but can be fatal when advanced. They can also cause significant morbidity due to the characteristic carcinoid syndrome. Somatostatin analogs continue to be the mainstay of treatment given their antiproliferative properties, as well as inhibitory effects on hormones that cause carcinoid syndrome. There have been several recent advances in the systemic therapy of these tumors including consolidation of somatostatin analogs as the cornerstone of therapy, completion of pivotal trials with mTOR inhibitors, and the establishment of novel approaches including peptide receptor radionuclide therapy and oral inhibitors of peripheral tryptophan hydroxylase in tumor and symptom control, respectively. In this review article, the recent advances are summarized and an updated approach to management is proposed.

Keywords:

• carcinoid
• everolimus
• lanreotide
• peptide receptor radionuclide therapy
• neuroendocrine
• octreotide
• telotristat

Financial & competing interests disclosure

A Dasari receives research funding from Novartis, Ipsen and is a consultant for Novartis, Ipsen. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.