Refractory carcinoid syndrome: a review of treatment options

Abstract

Carcinoid syndrome (CSy) is a constellation of symptoms that may commonly present in patients with well differentiated neuroendocrine tumors (NETs), with somatostatin analogs (SSAs) being the first-line option for symptom management. However, symptomatic progression eventually occurs and in this scenario of a refractory CSy; several treatment options have been studied such as dose escalation of SSA, interferon and liver-directed therapies. Nevertheless, recent phase III trials have contributed to the understanding and management of this condition. We performed a comprehensive review of interventional studies examining refractory CSy to provide the evidence for current treatment options and propose a treatment sequence.

Keywords: carcinoid syndrome; neuroendocrine neoplasm; somatostatin; systematic review.

Conflict of interest statement

Conflict of interest statement: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Rachel Riechelmann has received consultancy fees and honoraria from lectures from Novartis and Ipsen. Frederico Costa and Juliana Rego have received honoraria from lectures from Novartis.

Figures

Figure 1.

Refractory carcinoid syndrome: our proposal for a treatment sequence. SSA, somatostatin analog; NET, neuroendocrine tumors; PRRT, peptide receptor radionuclide therapy.