title: 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.
authors.affiliation: Instituto do Câncer do Estado de São Paulo,Universidade de São Paulo, Ave. Dr Arnaldo, 251, São Paulo, SP - Brazil.
authors.forename: Rachel P
authors.initials: RP
authors.lastname: Riechelmann
authors.affiliation: Department of Radiology and Oncology, Instituto do Câncer do Estado de São Paulo, São Paulo, Brazil.
authors.forename: Allan A
authors.initials: AA
authors.lastname: Pereira
authors.affiliation: Hospital Universitário Onofre Lopes, Universidade Federal do Rio Grande do Norte, Natal, Brazil.
authors.forename: Juliana F M
authors.initials: JF
authors.lastname: Rego
authors.affiliation: Hospital Sírio-Libanês, São Paulo, Brazil.
authors.forename: Frederico P
authors.initials: FP
authors.lastname: Costa
keywords: carcinoid syndrome; neuroendocrine neoplasm; somatostatin; systematic review